Tuberculous Otomastoiditis in Children Anne M. MacAdam, MD, Thomas Rubio, MD
\s=b\ We report three cases of tuberculous otomastoiditis in children. Review of the literature, with particular emphasis on the ten other cases reported since 1960, discloses that this form of tuberculosis is most frequent in infants and young children. All too often, the diagnosis is made too late, with resulting complications such as irreversible hearing loss and facial nerve paralysis. Antituberculous medication and surgical drainage controlled the infection in all 13 cases diagnosed since 1960. Tests for tuberculosis in children with chronic ear infection should be mandatory, but periodic testing of all children is recommended. (Am J Dis Child 131:152-156, 1977)
still remains the most notifiable infectious cause of death in the United States.1 The prevalence of the disease is par¬ ticularly high among certain popula¬ tion groups in Oklahoma. At the Uni¬ versity of Oklahoma Health Sciences Center, most of the cases are observed in American Indian and black pa¬ tients. Otomastoiditis is an extremely rare complication of tuberculosis today. To our knowledge, only ten cases in chil¬ dren have been reported since 1960. In the last five years three cases of tuberculous otitis media have been observed at the Children's Memorial Hospital of the University of Okla¬ homa Health Sciences Center. We will describe these three patients in detail, review the literature, and comment on the epidemiology, pathogenesis, treat¬ ment, and prevention of the disease.
Tubercul osis important
REPORT OF CASES Case 1.—A 5-year-old, Spanish-American girl was referred to Children's Memorial Hospital in October 1967 with a history of
From the Department of Pediatrics, Children's Memorial Hospital, University of Oklahoma Health Sciences Center; and the Oklahoma Department of Institutions, Social and Rehabilitative Services, Oklahoma City (Dr MacAdam). Dr Rubio is now with the Department of Pediatrics, Eastern Virginia Medical School, and Children's Hospital of the King's Daughters, Norfolk.
Reprint requests to Department of Pediatrics, Eastern Virginia Medical School, Children's Hospital of the King's Daughters, Norfolk, VA 23507
(Dr Rubio).
right ear pain, pharyngitis, and green, occasionally bloody, ear drainage that had persisted for one year despite antibiotic treatment.
She
was
admitted to the
hospital in March 1968 and tonsillectomy and adenoidectomy were performed be¬ cause of continued ear drainage. At admis¬ sion she had bilateral tympanic membrane perforations and bilateral cervical lym¬ phadenopathy, but was otherwise in good health. Mastoid roentgenograms showed decreased aeration bilaterally. Tonsils and adenoids were found to be very small at the time of surgery, but were removed despite their small size. Microscopic examination of the tonsils showed granulomatous inflammation and caseous necrosis. No acid-fast bacilli (AFB) were seen and no cultures for AFB were done. Following this report, further studies were done. The chest roentgenogram showed right hilar calcification. A skin test for tuberculosis with 1 tuberculin unit PPD showed 20 mm of induration at 48 hours. Culture of the ear drainage was positive for Mycobacterium tuberculosis. Cultures of the bone marrow, throat, urine, and gastric washings were all negative for AFB. Public health service records subsequent¬ ly disclosed that the patient's mother had been hospitalized in August 1963 with moderately advanced, active pulmonary tuberculosis and had left the hospital against medical advice while her disease was still active. The patient had had a positive tine test in October 1965 (two years prior to the present admission) and isoniazid therapy was begun. In July 1968, after the diag¬ nosis of tuberculous otitis was confirmed, the patient was started on a regimen of isoniazid, streptomycin, and aminosalycylic acid. Streptomycin was administered
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for one month. The aminosalycylic acid was continued until February 1970, and the isoniazid was given until February 1971. The patient did well until early 1970, when mild hearing loss was noted. By May 1970 a serous otitis and a 40-dB air-bone gap had developed. Ear culture in July 1970 grew Pseudomonas. Infection recurred several times over the next two years, necessitat¬ ing two myringotomies and leading to progressive retraction of the tympanic membranes and hearing loss. Repeated culture and smear for AFB, done in July 1972, were negative. When she was last seen in December 1972, she was doing well generally but had persistent, severe, bilat¬ eral hearing loss. Case 2.—A 3V2-year-old, Spanish-Amer¬ ican boy was admitted to Children's Memo¬ rial Hospital Aug 17, 1972, because of recurrent bilateral otitis media. His illness dated back to 14 months of age, when he developed a cough without fever that persisted for 22 days. This was followed by fever and copious white drainage from the ears approximately two weeks later. De¬ spite antibiotic therapy the patient's ear drainage persisted for more than one month, and recurred frequently during the next two years. He was first seen in our outpatient department on July 6, 1972. Cultures were obtained and a ten-day course of ampicillin sodium was started. Cultures were negative. Mastoid films were compatible with chronic mastoiditis. A 5-unit PPD tuberculin skin test applied on Aug 10 showed a 12-mm induration when it was read on Aug 17. Physical examination at the time of admission disclosed purulent-whitish drainage from both ears and bilateral tympanic membrane perforations. The lungs were clear and the child was other¬ wise healthy. Acid-fast bacilli were seen on a smear of the right ear drainage. Cultures of the ear drainage and of gastric washings were positive for M tuberculosis, but urine cultures were negative. The chest roent¬ genogram showed a left apical lesion that was compatible with the diagnosis of tuberculosis. During the years 1963 through 1965, the patient's father had been hospitalized for several months for active pulmonary tuber¬ culosis. He had been checked regularly and was quite sure that his disease was inactive at the time of questioning. The patient was given an 18-month course of isoniazid and aminosalycylic acid. When the patient was last seen in December 1973, the affected ear was dry, but a large perforation was evident on the tympanic membrane in the left ear and the ossicles were eroded. The mother reported that the child did not hear well. An
audiogram on Dec 4, 1973, showed a 40% to 50% hearing loss bilaterally. Case 3.-A 3-year-old, American Indian boy was referred to the Children's Memo¬ rial Hospital on Aug 29, 1972, with a history of posterior cervical lymphadeno¬ pathy and left ear drainage of eight months' duration. In May, he had begun to complain of abdominal pain, which pro¬ gressed in early July to generalized malaise and anorexia, weight loss, cough productive of yellow sputum, and persist¬ ent low-grade fever. Two weeks prior to admission facial paralysis on the left side
developed. Physical
examination
on
admission dis¬
cachectic, chronically ill child with ptosis of the right eyelid and purulent closed
a
drainage from
the left ear. Exudates were the tonsils. The right tympanic membrane was dull, but no exúdate was seen. Enlarged nodes were palpable in the posterior cervical and submandibular ar¬ eas. The lungs were clear. The liver was enlarged and tender. The chest roentgenogram showed dif¬ fuse, soft, nodular reticular infiltrates bilaterally that were consistent with miliary tuberculosis. Mastoid films showed scle¬ rosis on the left. A 1-unit PPD tuberculin skin test disclosed 20-mm of induration at 48 hours. Cultures of the urine, CSF, and bone marrow were negative for AFB. Mycobacterium tuberculosis was isolated in several gastric aspirates, and from the seen on
sputum. On Sept 13 the patient underwent simple
mastoidectomy and left facial nerve decompression. Microscopic examination of the excised tissue showed caseous debris and granulomatous material. Acid-fast organisms were also seen, and M tubercu¬ losis was cultured. Careful study of the home situation disclosed that the child had been cared for by a neighbor who had known active pulmonary tuberculosis. The child was treated with isoniazid, aminosalycylic acid,-and streptomycin. At follow-up examination on Jan 15,1973, only slight facial nerve dysfunction was noted. He had gained weight and exhibited good systemic recovery. Audiometry on May 14 showed a 60% hearing loss on the left.
REVIEW OF CASES SINCE 1960
To our knowledge, only ten cases of tuberculous otomastoiditis in children have been reported since I960.'-' The Table includes the clinical and labora¬ tory findings and the results of treat¬ ment in these ten cases. The age of the patients ranged from 6 weeks to 8 years. Six patients were less than 2
years old when the
diagnosis
was
made. There were seven boys and three girls. Most of the patients were black, American Indian, or Mexican, and most had been exposed to infected individuals within the immediate family. Bilateral disease was reported in half of the cases, and clinical or roentgenographic evidence of mastoiditis almost always was present. In four cases there was peripheral paral¬ ysis of the seventh cranial nerve. Pulmonary involvement was found frequently, and meningitis was pres¬ ent in two patients. Other manifesta¬ tions of severe active tuberculous disease included hepatosplenomegaly, skin abscesses, cervical lymphadeno¬ pathy, and postauricular fistulas. The diagnosis was confirmed by cultures in the majority of reported cases. In one case the tuberculin skin test was positive but no cultures were reported. The skin test was negative in one case, even though the cultures from the ear drainage, cervical node, and gastric washings grew M tubercu¬ losis. Treatment of most of the cases included isoniazid, aminosalycylic acid, and streptomycin. Most patients also required surgical drainage of the mastoid or of the cervical or preauri¬ cular lymph nodes. Some patients have been followed up for as long as three years. In most cases the drain¬ age decreased considerably or disap¬ peared. Facial paralysis, however, remained unaltered in three cases and follow-up data were not given for the fourth case. Persistent hearing loss was reported in some instances. Our three patients are similar to those previously reported with respect to age, ethnic background, and source of exposure. All hadAoth mastoid and lung involvement, and one child had miliary disease. Only one required surgery, however. Perhaps the most striking and important difference is that the child with the facial nerve paralysis has almost completely recov¬ ered from this complication. Hearing loss has been severe in all three chil¬ dren. The CSF was examined only in one of our patients and it was normal. Of particular interest are the two newborn infants with congenital tu-
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berculosis of the middle ear reported by Poley and Wissler.3 In both infants ear drainage and cervical lymphade¬ nopathy developed by the sixth week of life. The tuberculin skin test was positive in both infants, but one of them had received BCG vaccine on the tenth day of life. In both cases granulomatous lesions were found in the lymph nodes. Acid-fast bacilli were found in the ear drainage. One infant had persistent facial paralysis. Both had normal chest roentgenograms. Antituberculous medication led to im¬ provement, but psychomotor retarda¬ tion was noted in both patients after nine and 12 months of follow-up, respectively. Endometritis was discov¬ ered in the mother of one of the infants. The mother of the other infant had active pulmonary tubercu¬ losis. These two children exhibited several signs and symptoms not seen in our three cases, probably because of the difference in age. COMMENT
Epidemiology In the years before the advent of antituberculous chemotherapy, the oc¬ currence of tuberculous otitis and mastoiditis was frequent and widely recognized."7 Numerous case reports and reviews appeared in the litera¬ ture. In 1915 Turner and Fraser" published a detailed study of the clin¬ ical and pathologic findings in tuber¬ culous otitis. At that time most of the cases of middle ear drainage in chil¬ dren under 1 year of age were tuber¬ culous, while only a small percentage of cases in children of all ages were attributed to tubercle bacilli. The authors attributed the very high prev¬ alence in infancy to the use of unsterilized milk contaminated with M tuberculosis var bovis. In 1921 Leegaard" described 20 cases of otitis associated with M tuberculosis var hominis. Fourteen (70%) of these were in children under 15 years, but only one child was under 1 year of age. These figures correlated well with the incidence of skin test conversion in schoolchildren at the time. In 1942, in a review of the world literature, Proctor and Lindsay111 found that the incidence of tubercu-
losis in cases of chronic otitis media varied from 1.3% to 15.4% (average, 2.7%) in a total of 8,555 cases. These authors also reported a series of eight cases seen at the University of Chicago Clinics in the 1930s; patients ranged in age from 22 months to 8 years. They noted that "nearly all persons become infected with tuber¬ culosis in infancy or childhood...." In recent years the number of cases of tuberculous otitis or otomastoiditis appears to have dropped greatly, and children are not uniformly infected with tubercle bacilli in infancy.11 12 In a general analysis of case distribution, the US Department of Health, Educa¬ tion, and Welfare has found that the incidence of tuberculosis is high in the following locations: (1) large cities, especially low-income areas with crowded conditions; (2) many areas along the eastern coast and the Mexican border; (3) in the Appala¬ chian mountains; and (4) in areas with large American Indian populations. The statistics for the state of Okla¬ homa also show a gradual decrease in the incidence of tuberculosis compar¬ able to that in the nation as a whole.13 Yet the incidence in the American Indian population in Oklahoma in 1971 was still 64.7/100,000. Most of these cases of tuberculous otomastoiditis that have been re¬ ported in the past ten years have been from countries other than the United States. Three of the cases were from South Africa. All of these children were from the Bantu tribe, a group with a very high prevalence of tuber¬ culosis.1 Three others were from Mex¬ ico City. Only two patients were from the United States. One was a 19month-old black girl front Philadel¬ phia whose mother had a history of active tuberculosis.1 The other was a 3month-old white boy from St. Louis.1 All three patients whose conditions were diagnosed in our hospital were Indian of Mexican-American origin. Although tuberculosis in childhood is relatively rare today, tuberculous otitis or otomastoiditis seems to be more common among infants and young children than in older persons. Onset of the disease occurred within the first 2 years of life in eight of the 13 cases diagnosed since 1960. The
VII Nerve
Age/Sex 3yr/M
Location
Paralysis
Bilaterally
No
8yr/M
Right
2yr/M
Right
No
6yr/F
Right
No
4yr/F
Bilaterally
16 mo/F
Bilaterally
18mo/M
Right
No
3 mo/M
Left
No
6 wk/M
Right (?)
6wk/M
Right
No
5yr/F
Bilaterally
No
3yr/M
Bilaterally
No
3yr/M
Left
three cases described in detail by Turner and Frasers were all infants with advanced generalized disease.
Pathologic and Clinical Manifestations series of 60 cases (of which 51 children) reviewed by Turner and Fraser," 92% (55) of the patients had painless watery otorrhea and 95% (57) had enlarged lymph nodes. Laby¬ rinthine involvement was found in 53% (32). M'Cart" found that as many as 15% (7) of the 46 children in his series had ear pain at the onset of the disease; 94% (41) also had enlarged periauricular nodes, and 35% (16) had labyrinthine involvement. Leegaard" challenged the contention that pain¬ less otorrhea was a hallmark of the disease, stating that 40% (10) to 50% (13) of his 25 patients had some ear pain. While Turner and Fraser and M'Cart found a fairly high prevalence In
a
were
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Summary of 13 Cases* of Tuberculous Otomastoiditis Reported Since Other Manifestations Periorbital abscesses, corneal ulcération
Deafness,
Chest Roentgenogran Normal
retroauricular fistula Infiltrate Infiltrate
Retroauricular fistula, pulmonary disease, swollen knee Pulmonary disease,
MMIary
meningitis
INH
Mastoid
Follow-up, Duration/Results 3wk,
R mastoid
6 mo,
Surgery
Strep
Tine
improved
Tine
facial paralysis
Tine
R mastoid
Improved
R ear,
3yr, improved
lung
R mastoid
PPD PPD
Normal
Patch
7yr, postmenlngitls sequelae 3yr, Improved 2 mo, i mproved
R mastoid node
"Treated"
MMIary
MMIary
positive L cervical node
positive
1 yr,
improved
positive (BCG)
preauricular lymphadenitis
Cervical lymphadenitis,
Normal
Moro
Node
Chronic tonsillitis
Calcifi¬ cation
PPD
Tonsll-
Pulmonary disease
Infiltrate
PPD
splenomegaly
malnutrition
Improved
facial paralysis
Pulmonary disease,
severe
deafness (R) 1-2 yr,
Mastoid
"Treated"
Normal
Pulmonary disease,
Improved,
positive
Possible meningitis, corneal opacities
cervical mass, retinal lesion Cervical lymphadenitis,
PAS
positive
Pulmonary disease
TB
Therapyt Skin
positive Normal
Pulmonary disease
1960
facial paralysis 15 mo,
draining ear lymphadenopathy 3yr,
positive lectomy
positive
chronic otitis hearing loss
18 mo,
¡mproved hearing loss 1yr, improved bilateral hearing loss, slight facial nerve paralysis
positive
Miliary
L mastoid
PPD
positive
Ten previously reported cases plus our three cases (the last three listed in the Table) seen at Oklahoma Children's Memorial Hospital. t INH indicates isoniazid; PAS, aminosalycylic acid; strep, streptomycin; +, therapy given.
_
*
of facial
paralysis in their patients (45% (27) and 30% (14), respectively), Leegaard found none in his 25 cases. Leegaard considered this difference to be related to the severity of the
disease. Another characteristic of tubercu¬ lous otitis, and a severe problem, is early hearing loss. In 1960 Craig1' studied audiograms of eight children with tuberculous otitis. There was definite hearing loss, ranging from 30 to 90 dB in the speech frequencies in all cases. The most severe hearing losses occurred in patients who did not receive treatment (mastoidectomy, or mastoidectomy and streptomycin therapy) within a few weeks of the onset of symptoms. Yet even the child most promptly treated (nine days after the onset of symptoms) had a 50dB loss in the affected ear. All of these children are doing well otherwise, with no evidence of active disease.
Wallner,7 in a study of primarily adult patients, found that the hearing loss usually occurred very early in the disease, and often preceded any other symptoms. The slow development of deafness in one of our patients (case
1), however, may be an indication that
hearing loss
is
extremely variable.
Pathogenesis The pathogenesis of tuberculous otitis has been a matter of debate since the time of its recognition as a distinct entity."1"1'1 Five possible routes of infection have been de¬ scribed: (1) extension through the Eustachian tubes by droplets; (2) hematogenous spread; (3) infection via tympanic membrane perforation; (4) direct extension along the mucosal lining of the tube; and (5) direct extension from intracranial lesions. In congenital tuberculosis, endometritis of the mother is probably the most
common source
the
of contamination of
offspring. Treatment
The development and use of effec¬ tive antituberculous drugs has sharply altered the course and prognosis of tuberculosis in general. Leegaard1' was impressed by the very slow healing that he found in his patients after mastoidectomy, the process taking six months to a year or longer in some. The eight patients reviewed by Craig1' all had a protracted course with many complications. Most of them underwent only mastoidectomy, although three received streptomycin at some time during the clinical course. The clinical course in these children was not greatly altered. In 1964 Harbert and Riordan1" reviewed four patients with chronic otitis that were treated with mastoidectomy fol¬ lowed by chemotherapy after several
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years of otorrhea and hearing loss. All of these patients showed rapid clear¬ ance of drainage and greatly im¬
proved wound healing after antituber¬ culous drugs were started. Hearing, however, did not improve in any of these patients.
In 1953 Wallner addressed himself specifically to the question of the effectiveness of chemotherapy in al¬ tering the course of tuberculous otitis. He found that, with drugs, drainage ceased very rapidly and the ear lesions healed without the complications of meningitis or bone involvement. He also noted, however, that there was no improvement in hearing even when the disease was diagnosed very early. It would seem, then, that chemo¬ therapy has shortened the course and severity of tuberculous otitis, as it has other forms of tuberculosis. It has not,
however, altered the prevalence
or
severity of the hearing loss that accompanies the otitis, at least in cases where deafness occurs early in
of the illness. Nor does it capable of reversing the facial nerve paralysis in the cases previously reported. Fields1" found no the
course
seem
to be
improvement in the paralysis in his patient
facial as
nerve
long
years after the onset of the
as
12
disease,
the fact that the child had received antituberculous drug therapy with rapid resolution of the disease process. Our third patient (case 3) seems to be a fortunate exception to this general trend. In addition to pre¬ scribing antituberculous medications, surgical drainage should be done when needed. The combination of medical and surgical treatment has controlled the infection in all the cases diagnosed since 1960, but in some instances secondary infection with Pseudomo¬ nas or other organisms has been observed.
despite
Conclusion
How, then,
can we
improve
treat¬
of these children? Because tuberculosis is a rare cause of otitis today, the diagnosis usually is not even considered until the child has undergone several courses of anti¬ biotic therapy without improvement. While hearing loss may be evident quite early in the older child, it is frequently missed in the infant, espement
if the lesion is unilateral.17 Any child with chronic suppurative disease of the middle ear, particularly with multiple perforations of the tympanic membrane, should be tested for tuber¬ culosis. We may at least prevent the complication of facial nerve paralysis by early treatment. Because the hear¬ ing loss usually occurs early in the disease process, it seems that the only really effective treatment of this is prevention. The epidemiologie data that we have presented point out the need for greater diligence in looking for tuberculous infection in the Indian and black populations. Perhaps, a more thorough search for tuberculosis in these groups will eliminate this rare but serious form of the disease. Peri¬ odic tuberculin skin testing should detect most of the children with primary tuberculosis.
cially
Nonproprietary Name and Trademarks of Drug Ampicillin sodium-Alpen-N, Amcill-S, Omnipen-N, Penbritin-S, Polycillin-N, Principen/N, Totacillin-N.
References 1. Wolfowitz BL: Tuberculous mastoiditis. Arch Otolaryngol 95:109-113, 1972. 2. Smoler J, Pinto SL, Vivar G, et al: Tuberculous otitis media. Laryngoscope 79:488-493, 1969. 3. Marlowe FI: Primary tuberculous otomastoiditis. Ann Otol Rhinol Laryngol 81:288-290, 1972. 4. Saltzman SJ, Feigin RD: Tuberculous otitis media and mastoiditis. J Pediatr 79:1004-1006, 1971. 5. Poley JR, Wissler H: Prim\l=a"\re, konnatale Mittelohrtuberkulose. Helv Paediatr Acta 19:587\x=req-\ 596, 1964. 6. Baar HS, Evans R: Primary tuberculous complex of the middle ear. J Laryngol 40:456-466,
1925. 7. Wallner LF:
Tuberculous otitis media. Laryngoscope 63:1058-1077, 1953. 8. Turner AL, Fraser JS: Tuberculosis of the middle-ear cleft in children: A clinical and pathological study. J Laryngol Otol 30:15-247, 1915. 9. Leegaard F: Tuberculosis of the middle ear: Laryngoscope 31:374-379, 1921. 10. Proctor B, Lindsay JR: Tuberculosis of the ear. Arch Otolaryngol 35:221-249, 1942. 11. Reported Tuberculosis Data 1970. US Department of Health, Education, and Welfare, Public Health Service, 1972. 12. 1971 Tuberculosis Statistics: States and Cities. US Department of Health, Education, and
Welfare, Health Services and Mental Health Administration, 1972. 13.
Reported
Tuberculosis Data: Oklahoma.
Oklahoma State Department of Health, 1972.
14. M'Cart HWD: Tuberculous disease of the middle ear. J Laryngol 40:456-466, 1925. 15. Craig DH: Tuberculous mastoiditis: A review of eight cases. J Laryngol 76:623-638, 1962. 16. Harbert F, Riordan D: Tuberculosis of the middle ear. Laryngoscope 74:198-204, 1964. 17. Lincoln EM, Sewell EM: Tuberculosis of the mouth and upper respiratory tract, in Tuberculosis in Children. New York, McGraw-Hill Book Co Inc, 1963, pp 216-223.
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\s=b\ We report three cases of tuberculous otomastoiditis in children. Review of the literature, with particular emphasis on the ten other cases reported since 1960, discloses that this form of tuberculosis is most frequent in infants and young children. All too often, the diagnosis is made too late, with resulting complications such as irreversible hearing loss and facial nerve paralysis. Antituberculous medication and surgical drainage controlled the infection in all 13 cases diagnosed since 1960. Tests for tuberculosis in children with chronic ear infection should be mandatory, but periodic testing of all children is recommended. (Am J Dis Child 131:152-156, 1977)
still remains the most notifiable infectious cause of death in the United States.1 The prevalence of the disease is par¬ ticularly high among certain popula¬ tion groups in Oklahoma. At the Uni¬ versity of Oklahoma Health Sciences Center, most of the cases are observed in American Indian and black pa¬ tients. Otomastoiditis is an extremely rare complication of tuberculosis today. To our knowledge, only ten cases in chil¬ dren have been reported since 1960. In the last five years three cases of tuberculous otitis media have been observed at the Children's Memorial Hospital of the University of Okla¬ homa Health Sciences Center. We will describe these three patients in detail, review the literature, and comment on the epidemiology, pathogenesis, treat¬ ment, and prevention of the disease.
Tubercul osis important
REPORT OF CASES Case 1.—A 5-year-old, Spanish-American girl was referred to Children's Memorial Hospital in October 1967 with a history of
From the Department of Pediatrics, Children's Memorial Hospital, University of Oklahoma Health Sciences Center; and the Oklahoma Department of Institutions, Social and Rehabilitative Services, Oklahoma City (Dr MacAdam). Dr Rubio is now with the Department of Pediatrics, Eastern Virginia Medical School, and Children's Hospital of the King's Daughters, Norfolk.
Reprint requests to Department of Pediatrics, Eastern Virginia Medical School, Children's Hospital of the King's Daughters, Norfolk, VA 23507
(Dr Rubio).
right ear pain, pharyngitis, and green, occasionally bloody, ear drainage that had persisted for one year despite antibiotic treatment.
She
was
admitted to the
hospital in March 1968 and tonsillectomy and adenoidectomy were performed be¬ cause of continued ear drainage. At admis¬ sion she had bilateral tympanic membrane perforations and bilateral cervical lym¬ phadenopathy, but was otherwise in good health. Mastoid roentgenograms showed decreased aeration bilaterally. Tonsils and adenoids were found to be very small at the time of surgery, but were removed despite their small size. Microscopic examination of the tonsils showed granulomatous inflammation and caseous necrosis. No acid-fast bacilli (AFB) were seen and no cultures for AFB were done. Following this report, further studies were done. The chest roentgenogram showed right hilar calcification. A skin test for tuberculosis with 1 tuberculin unit PPD showed 20 mm of induration at 48 hours. Culture of the ear drainage was positive for Mycobacterium tuberculosis. Cultures of the bone marrow, throat, urine, and gastric washings were all negative for AFB. Public health service records subsequent¬ ly disclosed that the patient's mother had been hospitalized in August 1963 with moderately advanced, active pulmonary tuberculosis and had left the hospital against medical advice while her disease was still active. The patient had had a positive tine test in October 1965 (two years prior to the present admission) and isoniazid therapy was begun. In July 1968, after the diag¬ nosis of tuberculous otitis was confirmed, the patient was started on a regimen of isoniazid, streptomycin, and aminosalycylic acid. Streptomycin was administered
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for one month. The aminosalycylic acid was continued until February 1970, and the isoniazid was given until February 1971. The patient did well until early 1970, when mild hearing loss was noted. By May 1970 a serous otitis and a 40-dB air-bone gap had developed. Ear culture in July 1970 grew Pseudomonas. Infection recurred several times over the next two years, necessitat¬ ing two myringotomies and leading to progressive retraction of the tympanic membranes and hearing loss. Repeated culture and smear for AFB, done in July 1972, were negative. When she was last seen in December 1972, she was doing well generally but had persistent, severe, bilat¬ eral hearing loss. Case 2.—A 3V2-year-old, Spanish-Amer¬ ican boy was admitted to Children's Memo¬ rial Hospital Aug 17, 1972, because of recurrent bilateral otitis media. His illness dated back to 14 months of age, when he developed a cough without fever that persisted for 22 days. This was followed by fever and copious white drainage from the ears approximately two weeks later. De¬ spite antibiotic therapy the patient's ear drainage persisted for more than one month, and recurred frequently during the next two years. He was first seen in our outpatient department on July 6, 1972. Cultures were obtained and a ten-day course of ampicillin sodium was started. Cultures were negative. Mastoid films were compatible with chronic mastoiditis. A 5-unit PPD tuberculin skin test applied on Aug 10 showed a 12-mm induration when it was read on Aug 17. Physical examination at the time of admission disclosed purulent-whitish drainage from both ears and bilateral tympanic membrane perforations. The lungs were clear and the child was other¬ wise healthy. Acid-fast bacilli were seen on a smear of the right ear drainage. Cultures of the ear drainage and of gastric washings were positive for M tuberculosis, but urine cultures were negative. The chest roent¬ genogram showed a left apical lesion that was compatible with the diagnosis of tuberculosis. During the years 1963 through 1965, the patient's father had been hospitalized for several months for active pulmonary tuber¬ culosis. He had been checked regularly and was quite sure that his disease was inactive at the time of questioning. The patient was given an 18-month course of isoniazid and aminosalycylic acid. When the patient was last seen in December 1973, the affected ear was dry, but a large perforation was evident on the tympanic membrane in the left ear and the ossicles were eroded. The mother reported that the child did not hear well. An
audiogram on Dec 4, 1973, showed a 40% to 50% hearing loss bilaterally. Case 3.-A 3-year-old, American Indian boy was referred to the Children's Memo¬ rial Hospital on Aug 29, 1972, with a history of posterior cervical lymphadeno¬ pathy and left ear drainage of eight months' duration. In May, he had begun to complain of abdominal pain, which pro¬ gressed in early July to generalized malaise and anorexia, weight loss, cough productive of yellow sputum, and persist¬ ent low-grade fever. Two weeks prior to admission facial paralysis on the left side
developed. Physical
examination
on
admission dis¬
cachectic, chronically ill child with ptosis of the right eyelid and purulent closed
a
drainage from
the left ear. Exudates were the tonsils. The right tympanic membrane was dull, but no exúdate was seen. Enlarged nodes were palpable in the posterior cervical and submandibular ar¬ eas. The lungs were clear. The liver was enlarged and tender. The chest roentgenogram showed dif¬ fuse, soft, nodular reticular infiltrates bilaterally that were consistent with miliary tuberculosis. Mastoid films showed scle¬ rosis on the left. A 1-unit PPD tuberculin skin test disclosed 20-mm of induration at 48 hours. Cultures of the urine, CSF, and bone marrow were negative for AFB. Mycobacterium tuberculosis was isolated in several gastric aspirates, and from the seen on
sputum. On Sept 13 the patient underwent simple
mastoidectomy and left facial nerve decompression. Microscopic examination of the excised tissue showed caseous debris and granulomatous material. Acid-fast organisms were also seen, and M tubercu¬ losis was cultured. Careful study of the home situation disclosed that the child had been cared for by a neighbor who had known active pulmonary tuberculosis. The child was treated with isoniazid, aminosalycylic acid,-and streptomycin. At follow-up examination on Jan 15,1973, only slight facial nerve dysfunction was noted. He had gained weight and exhibited good systemic recovery. Audiometry on May 14 showed a 60% hearing loss on the left.
REVIEW OF CASES SINCE 1960
To our knowledge, only ten cases of tuberculous otomastoiditis in children have been reported since I960.'-' The Table includes the clinical and labora¬ tory findings and the results of treat¬ ment in these ten cases. The age of the patients ranged from 6 weeks to 8 years. Six patients were less than 2
years old when the
diagnosis
was
made. There were seven boys and three girls. Most of the patients were black, American Indian, or Mexican, and most had been exposed to infected individuals within the immediate family. Bilateral disease was reported in half of the cases, and clinical or roentgenographic evidence of mastoiditis almost always was present. In four cases there was peripheral paral¬ ysis of the seventh cranial nerve. Pulmonary involvement was found frequently, and meningitis was pres¬ ent in two patients. Other manifesta¬ tions of severe active tuberculous disease included hepatosplenomegaly, skin abscesses, cervical lymphadeno¬ pathy, and postauricular fistulas. The diagnosis was confirmed by cultures in the majority of reported cases. In one case the tuberculin skin test was positive but no cultures were reported. The skin test was negative in one case, even though the cultures from the ear drainage, cervical node, and gastric washings grew M tubercu¬ losis. Treatment of most of the cases included isoniazid, aminosalycylic acid, and streptomycin. Most patients also required surgical drainage of the mastoid or of the cervical or preauri¬ cular lymph nodes. Some patients have been followed up for as long as three years. In most cases the drain¬ age decreased considerably or disap¬ peared. Facial paralysis, however, remained unaltered in three cases and follow-up data were not given for the fourth case. Persistent hearing loss was reported in some instances. Our three patients are similar to those previously reported with respect to age, ethnic background, and source of exposure. All hadAoth mastoid and lung involvement, and one child had miliary disease. Only one required surgery, however. Perhaps the most striking and important difference is that the child with the facial nerve paralysis has almost completely recov¬ ered from this complication. Hearing loss has been severe in all three chil¬ dren. The CSF was examined only in one of our patients and it was normal. Of particular interest are the two newborn infants with congenital tu-
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berculosis of the middle ear reported by Poley and Wissler.3 In both infants ear drainage and cervical lymphade¬ nopathy developed by the sixth week of life. The tuberculin skin test was positive in both infants, but one of them had received BCG vaccine on the tenth day of life. In both cases granulomatous lesions were found in the lymph nodes. Acid-fast bacilli were found in the ear drainage. One infant had persistent facial paralysis. Both had normal chest roentgenograms. Antituberculous medication led to im¬ provement, but psychomotor retarda¬ tion was noted in both patients after nine and 12 months of follow-up, respectively. Endometritis was discov¬ ered in the mother of one of the infants. The mother of the other infant had active pulmonary tubercu¬ losis. These two children exhibited several signs and symptoms not seen in our three cases, probably because of the difference in age. COMMENT
Epidemiology In the years before the advent of antituberculous chemotherapy, the oc¬ currence of tuberculous otitis and mastoiditis was frequent and widely recognized."7 Numerous case reports and reviews appeared in the litera¬ ture. In 1915 Turner and Fraser" published a detailed study of the clin¬ ical and pathologic findings in tuber¬ culous otitis. At that time most of the cases of middle ear drainage in chil¬ dren under 1 year of age were tuber¬ culous, while only a small percentage of cases in children of all ages were attributed to tubercle bacilli. The authors attributed the very high prev¬ alence in infancy to the use of unsterilized milk contaminated with M tuberculosis var bovis. In 1921 Leegaard" described 20 cases of otitis associated with M tuberculosis var hominis. Fourteen (70%) of these were in children under 15 years, but only one child was under 1 year of age. These figures correlated well with the incidence of skin test conversion in schoolchildren at the time. In 1942, in a review of the world literature, Proctor and Lindsay111 found that the incidence of tubercu-
losis in cases of chronic otitis media varied from 1.3% to 15.4% (average, 2.7%) in a total of 8,555 cases. These authors also reported a series of eight cases seen at the University of Chicago Clinics in the 1930s; patients ranged in age from 22 months to 8 years. They noted that "nearly all persons become infected with tuber¬ culosis in infancy or childhood...." In recent years the number of cases of tuberculous otitis or otomastoiditis appears to have dropped greatly, and children are not uniformly infected with tubercle bacilli in infancy.11 12 In a general analysis of case distribution, the US Department of Health, Educa¬ tion, and Welfare has found that the incidence of tuberculosis is high in the following locations: (1) large cities, especially low-income areas with crowded conditions; (2) many areas along the eastern coast and the Mexican border; (3) in the Appala¬ chian mountains; and (4) in areas with large American Indian populations. The statistics for the state of Okla¬ homa also show a gradual decrease in the incidence of tuberculosis compar¬ able to that in the nation as a whole.13 Yet the incidence in the American Indian population in Oklahoma in 1971 was still 64.7/100,000. Most of these cases of tuberculous otomastoiditis that have been re¬ ported in the past ten years have been from countries other than the United States. Three of the cases were from South Africa. All of these children were from the Bantu tribe, a group with a very high prevalence of tuber¬ culosis.1 Three others were from Mex¬ ico City. Only two patients were from the United States. One was a 19month-old black girl front Philadel¬ phia whose mother had a history of active tuberculosis.1 The other was a 3month-old white boy from St. Louis.1 All three patients whose conditions were diagnosed in our hospital were Indian of Mexican-American origin. Although tuberculosis in childhood is relatively rare today, tuberculous otitis or otomastoiditis seems to be more common among infants and young children than in older persons. Onset of the disease occurred within the first 2 years of life in eight of the 13 cases diagnosed since 1960. The
VII Nerve
Age/Sex 3yr/M
Location
Paralysis
Bilaterally
No
8yr/M
Right
2yr/M
Right
No
6yr/F
Right
No
4yr/F
Bilaterally
16 mo/F
Bilaterally
18mo/M
Right
No
3 mo/M
Left
No
6 wk/M
Right (?)
6wk/M
Right
No
5yr/F
Bilaterally
No
3yr/M
Bilaterally
No
3yr/M
Left
three cases described in detail by Turner and Frasers were all infants with advanced generalized disease.
Pathologic and Clinical Manifestations series of 60 cases (of which 51 children) reviewed by Turner and Fraser," 92% (55) of the patients had painless watery otorrhea and 95% (57) had enlarged lymph nodes. Laby¬ rinthine involvement was found in 53% (32). M'Cart" found that as many as 15% (7) of the 46 children in his series had ear pain at the onset of the disease; 94% (41) also had enlarged periauricular nodes, and 35% (16) had labyrinthine involvement. Leegaard" challenged the contention that pain¬ less otorrhea was a hallmark of the disease, stating that 40% (10) to 50% (13) of his 25 patients had some ear pain. While Turner and Fraser and M'Cart found a fairly high prevalence In
a
were
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Summary of 13 Cases* of Tuberculous Otomastoiditis Reported Since Other Manifestations Periorbital abscesses, corneal ulcération
Deafness,
Chest Roentgenogran Normal
retroauricular fistula Infiltrate Infiltrate
Retroauricular fistula, pulmonary disease, swollen knee Pulmonary disease,
MMIary
meningitis
INH
Mastoid
Follow-up, Duration/Results 3wk,
R mastoid
6 mo,
Surgery
Strep
Tine
improved
Tine
facial paralysis
Tine
R mastoid
Improved
R ear,
3yr, improved
lung
R mastoid
PPD PPD
Normal
Patch
7yr, postmenlngitls sequelae 3yr, Improved 2 mo, i mproved
R mastoid node
"Treated"
MMIary
MMIary
positive L cervical node
positive
1 yr,
improved
positive (BCG)
preauricular lymphadenitis
Cervical lymphadenitis,
Normal
Moro
Node
Chronic tonsillitis
Calcifi¬ cation
PPD
Tonsll-
Pulmonary disease
Infiltrate
PPD
splenomegaly
malnutrition
Improved
facial paralysis
Pulmonary disease,
severe
deafness (R) 1-2 yr,
Mastoid
"Treated"
Normal
Pulmonary disease,
Improved,
positive
Possible meningitis, corneal opacities
cervical mass, retinal lesion Cervical lymphadenitis,
PAS
positive
Pulmonary disease
TB
Therapyt Skin
positive Normal
Pulmonary disease
1960
facial paralysis 15 mo,
draining ear lymphadenopathy 3yr,
positive lectomy
positive
chronic otitis hearing loss
18 mo,
¡mproved hearing loss 1yr, improved bilateral hearing loss, slight facial nerve paralysis
positive
Miliary
L mastoid
PPD
positive
Ten previously reported cases plus our three cases (the last three listed in the Table) seen at Oklahoma Children's Memorial Hospital. t INH indicates isoniazid; PAS, aminosalycylic acid; strep, streptomycin; +, therapy given.
_
*
of facial
paralysis in their patients (45% (27) and 30% (14), respectively), Leegaard found none in his 25 cases. Leegaard considered this difference to be related to the severity of the
disease. Another characteristic of tubercu¬ lous otitis, and a severe problem, is early hearing loss. In 1960 Craig1' studied audiograms of eight children with tuberculous otitis. There was definite hearing loss, ranging from 30 to 90 dB in the speech frequencies in all cases. The most severe hearing losses occurred in patients who did not receive treatment (mastoidectomy, or mastoidectomy and streptomycin therapy) within a few weeks of the onset of symptoms. Yet even the child most promptly treated (nine days after the onset of symptoms) had a 50dB loss in the affected ear. All of these children are doing well otherwise, with no evidence of active disease.
Wallner,7 in a study of primarily adult patients, found that the hearing loss usually occurred very early in the disease, and often preceded any other symptoms. The slow development of deafness in one of our patients (case
1), however, may be an indication that
hearing loss
is
extremely variable.
Pathogenesis The pathogenesis of tuberculous otitis has been a matter of debate since the time of its recognition as a distinct entity."1"1'1 Five possible routes of infection have been de¬ scribed: (1) extension through the Eustachian tubes by droplets; (2) hematogenous spread; (3) infection via tympanic membrane perforation; (4) direct extension along the mucosal lining of the tube; and (5) direct extension from intracranial lesions. In congenital tuberculosis, endometritis of the mother is probably the most
common source
the
of contamination of
offspring. Treatment
The development and use of effec¬ tive antituberculous drugs has sharply altered the course and prognosis of tuberculosis in general. Leegaard1' was impressed by the very slow healing that he found in his patients after mastoidectomy, the process taking six months to a year or longer in some. The eight patients reviewed by Craig1' all had a protracted course with many complications. Most of them underwent only mastoidectomy, although three received streptomycin at some time during the clinical course. The clinical course in these children was not greatly altered. In 1964 Harbert and Riordan1" reviewed four patients with chronic otitis that were treated with mastoidectomy fol¬ lowed by chemotherapy after several
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years of otorrhea and hearing loss. All of these patients showed rapid clear¬ ance of drainage and greatly im¬
proved wound healing after antituber¬ culous drugs were started. Hearing, however, did not improve in any of these patients.
In 1953 Wallner addressed himself specifically to the question of the effectiveness of chemotherapy in al¬ tering the course of tuberculous otitis. He found that, with drugs, drainage ceased very rapidly and the ear lesions healed without the complications of meningitis or bone involvement. He also noted, however, that there was no improvement in hearing even when the disease was diagnosed very early. It would seem, then, that chemo¬ therapy has shortened the course and severity of tuberculous otitis, as it has other forms of tuberculosis. It has not,
however, altered the prevalence
or
severity of the hearing loss that accompanies the otitis, at least in cases where deafness occurs early in
of the illness. Nor does it capable of reversing the facial nerve paralysis in the cases previously reported. Fields1" found no the
course
seem
to be
improvement in the paralysis in his patient
facial as
nerve
long
years after the onset of the
as
12
disease,
the fact that the child had received antituberculous drug therapy with rapid resolution of the disease process. Our third patient (case 3) seems to be a fortunate exception to this general trend. In addition to pre¬ scribing antituberculous medications, surgical drainage should be done when needed. The combination of medical and surgical treatment has controlled the infection in all the cases diagnosed since 1960, but in some instances secondary infection with Pseudomo¬ nas or other organisms has been observed.
despite
Conclusion
How, then,
can we
improve
treat¬
of these children? Because tuberculosis is a rare cause of otitis today, the diagnosis usually is not even considered until the child has undergone several courses of anti¬ biotic therapy without improvement. While hearing loss may be evident quite early in the older child, it is frequently missed in the infant, espement
if the lesion is unilateral.17 Any child with chronic suppurative disease of the middle ear, particularly with multiple perforations of the tympanic membrane, should be tested for tuber¬ culosis. We may at least prevent the complication of facial nerve paralysis by early treatment. Because the hear¬ ing loss usually occurs early in the disease process, it seems that the only really effective treatment of this is prevention. The epidemiologie data that we have presented point out the need for greater diligence in looking for tuberculous infection in the Indian and black populations. Perhaps, a more thorough search for tuberculosis in these groups will eliminate this rare but serious form of the disease. Peri¬ odic tuberculin skin testing should detect most of the children with primary tuberculosis.
cially
Nonproprietary Name and Trademarks of Drug Ampicillin sodium-Alpen-N, Amcill-S, Omnipen-N, Penbritin-S, Polycillin-N, Principen/N, Totacillin-N.
References 1. Wolfowitz BL: Tuberculous mastoiditis. Arch Otolaryngol 95:109-113, 1972. 2. Smoler J, Pinto SL, Vivar G, et al: Tuberculous otitis media. Laryngoscope 79:488-493, 1969. 3. Marlowe FI: Primary tuberculous otomastoiditis. Ann Otol Rhinol Laryngol 81:288-290, 1972. 4. Saltzman SJ, Feigin RD: Tuberculous otitis media and mastoiditis. J Pediatr 79:1004-1006, 1971. 5. Poley JR, Wissler H: Prim\l=a"\re, konnatale Mittelohrtuberkulose. Helv Paediatr Acta 19:587\x=req-\ 596, 1964. 6. Baar HS, Evans R: Primary tuberculous complex of the middle ear. J Laryngol 40:456-466,
1925. 7. Wallner LF:
Tuberculous otitis media. Laryngoscope 63:1058-1077, 1953. 8. Turner AL, Fraser JS: Tuberculosis of the middle-ear cleft in children: A clinical and pathological study. J Laryngol Otol 30:15-247, 1915. 9. Leegaard F: Tuberculosis of the middle ear: Laryngoscope 31:374-379, 1921. 10. Proctor B, Lindsay JR: Tuberculosis of the ear. Arch Otolaryngol 35:221-249, 1942. 11. Reported Tuberculosis Data 1970. US Department of Health, Education, and Welfare, Public Health Service, 1972. 12. 1971 Tuberculosis Statistics: States and Cities. US Department of Health, Education, and
Welfare, Health Services and Mental Health Administration, 1972. 13.
Reported
Tuberculosis Data: Oklahoma.
Oklahoma State Department of Health, 1972.
14. M'Cart HWD: Tuberculous disease of the middle ear. J Laryngol 40:456-466, 1925. 15. Craig DH: Tuberculous mastoiditis: A review of eight cases. J Laryngol 76:623-638, 1962. 16. Harbert F, Riordan D: Tuberculosis of the middle ear. Laryngoscope 74:198-204, 1964. 17. Lincoln EM, Sewell EM: Tuberculosis of the mouth and upper respiratory tract, in Tuberculosis in Children. New York, McGraw-Hill Book Co Inc, 1963, pp 216-223.
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