[Deafness at Wiskott-Aldrich-syndrome].

INTRODUCTION

The Wiskott-Aldrich-syndrome belongs to the phakomatoses. The hereditary transmission happens x-chromosomal recessive in the domain of Xp 11.3-Xp 11.22. Clinical evidence for that is given by thrombocytopenia, an eczema and a weakness of the immune system with a subsequent increased risk of frequent infections and a predisposition for a malignancies. A further characteristic of that disease is recurrent otitis media as described by Aldrich in 1954.

PATIENT: The case--as described above--is about a 28-year-old male patient developing an acute deafness on the left ear while he has been suffering from a surditas on the right ear for 5 years. 8 years ago a splenectomy was made, because of persistent thrombocytopenia. An additional clinical characteristic was a hemiballism, arising after a thalamusbleeding 5 years ago, a both-sided vestibular failure concerning both that has been existing for 2 years, as well as a maculopathia with a highly reduced visus on both sides.

CONCLUSIONS

The deafness arising in patients suffering from the Wiskott-Aldrich-syndrome represents a so far not described symptom of that illness.