LATE-ONSET SENSORINEURAL HEARING-LOSS IN COFFIN-LOWRY-SYNDROME

The Coffin-Lowry syndrome (CLS) is a rare clinical entity where patien ts present with a characteristic facies. mental retardation and bone a bnormalities. So far about 60 cases have been reported. Sensorineural hearing loss is not a characteristic symptom and a late-onset hearing loss has not been reported so far. We report on 4 brothers with CLS, a ged 9-17 years, of whom 3 suffered from a severe sensorineural hearing loss with an onset during late childhood and adolescence after a norm al hearing during early childhood. CT scans revealed no cochlear abnor malities, The boys were successfully equipped with hearing aids. It is suggested that in all CLS patients the hearing ability should be exam ined during early infancy and retested regularly in cases with normal hearing as these cases indicate that in CLS a late-onset hearing loss is possible during late childhood and adolescence.