British Jottmal of Dermatology (1978) 99, 325.
Case Reports
Atopic dermatitis and congenital deafness F.SCHULTZ LARSEN, P.VASE AND H.SCHMIDT Departments of Dermatology and Otolaryngology, Odense University Hospital, Odense, Denmark Accepted for publication 3 March 1978
SUMMARY
In a family with perceptive, non-progressive hearing loss several ofthe members suffered from atopic dermatitis. The proband had a severe atopic dermatitis and an extremely high IgE value. Some of the family members suifered from atopic dermatitis, others from deafness, and some from both diseases. The co-existence of these two disorders has been previously described in two families. Atopic dermatitis and perceptive, non-progressive congenital deafness might be genetically associated.
A number of hereditary syndromes with dermatological and auditory manifestations have previously been published (Reed et al., 1967). Atopic dermatitis accompanied by congenital deafness has been reported in two families (Koningsmark et al, 1968; Frentz et al.y 1976). We report our findings in a family where some of the members suffer from atopic eczema, others from deafness and some from both diseases. MATERIAL AND METHOD
The material consists of 23 family members representing four generations. Eleven of them were examined, while information about the other family members has been obtained through questionnaires (Fig. i). The otological examination was supplemented with sound and speech audiometric tests, tympanometric tests and stapedius reflex tests. Furthermore) the proband was examined by a tone decay test, vestibular test and X-ray examination of temporal bones including tomography. Laboratory investigations included haemoglobin, leukocytes, differential count, sedimentation rate, circulating eosinophils and immunoglobulins. CASE REPORT
The proband is a 15-year-oId boy who has suffered from atopic dermatitis since the age of 9 months. At first the eczema appeared on the cheeks; later on it was localized to the arms and legs especially involving the flexural areas. From early childhood he also suffered from asthma and allergic rhinitis. His eczema as well as the asthma and the rhinitis exacerbated during spring. 0007-0963/78/0900-0000 $02.00 © 1978 British Association of Dennatologists
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proband a 3 male/female affected with atopic dermatitis I] O male/female affected with congenital deafness U G male/female investigated FIGURE I. Pedigree of the family.
From the age of 3 years hearing loss was suspected. However, he was not audiologically examined until he was ii-years-old. Before that time he had stayed at a children's psychiatric hospital for almost 3 years on the assumption that he was mentally retarded as he had a speech impediment. He had a typical atopic dermaticis, with eczematous and lichenified lesions involving the hands, wrists, forearms, ankles and legs. Furthermore, excoriated papules were ohserved around the neck and on the upper trunk. There were fissures around the mouth and palmar keratosis with accentuated skin lines. The audiometric test revealed a pronounced bilateral perceptive hearing loss (Fig. 2) with a 'basin curve' and small conductive element. Otoscopy showed a bilateral external otitis and a slightly thickened tympanum in the right ear. The tympanometric test showed a slight low-pressure in both middle ears. The other otological examinations were normal. Laboratory investigations. Circulating eosinophils 867 mm " •* (normal range 0-450), IgG, IgM and IgA normal, IgE 30000 units/ml (normal range 0-150 units/mi, i unit—2 3 ng/ml). Intracutaneous skin tests to common allergens were positive to the following antigens: house dust mite, birch, alder, hazel. Allergen specific IgE in serum (RAST, Pharmacia): house dust mite 4, birch 4, alder 4, hazel 3, dog 2, horse 2. Allergen specific IgE in serum measured by aluminium radio-allergosorbent test (Weeke, 1978): house dust mite 2319, birch 26, alder 17, hazel 7, dog 2, horse 2. Chromosome study. Normal complement 46, XY. Chromatographic study of amino acid content in the urine showed normal levels. His mental and linguistic development started to make progress after the beginning of audiological treatment and speech therapy. Intensive house dust clearance in the home, disodium cromoglycate given nasally and treatment with topical steroids improved the alopic diseases. D3ring the improvement the IgE level dropped to 6800 units/ml. Family history and findings
The numerical order of the 11 examined family members is shown in Fig. i. No. 3 (i6-years-old). During infancy he had dry skin and itching eczema in the flexures. He still shows keratosis pilaris on both legs. A perceptive hearing loss was found at the age of 10 years with
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FIGURE 2. Audiogram of the proband. Symbols: air conducLion, right car ( j ); left car (x X). Bone conduction, right ear (J ]); left ear ([ [). Stapedius refleXj sound in right ear C-^), sound in left ear (