Autoimmune sensorineural hearing loss in young patients: an exploratory study.

OBJECTIVES: P0 protein is expressed exclusively in myelinating Schwann cells of the peripheral nervous system. In a previous study from our laboratory, 27% of patients with sensorineural hearing loss (SNHL) had antibodies to P0 protein in their serum. The purpose of the present exploratory study was to examine the relationship between the clinical presentation of SNHL among children and young adults (age range, 5-30 y) and the presence of serum anti-P0 antibodies. STUDY DESIGN: The data were collected by retrospective questionnaires from Belgian otolaryngologists. METHODS: Patients were divided for comparison into two groups according to the presence or absence of anti-P0 antibodies. RESULTS: Analyses of clinical data and audiometric results indicated that a progressive hearing loss was more frequently recorded in the patients in the anti-P0 antibody-positive group (82% [14 of 17]) than in those in the anti-P0 antibody-negative group (35% [6 of 17]) (P <.005). CONCLUSIONS: Thus, in the age group in the present study, autoimmune SNHL (as measured in the present study by the presence of anti-P0 antibodies) is more frequently associated with progressive than with sudden hearing loss. The implications of this finding for preventive screening of hearing loss in children and young adults are discussed