Short-latency brainstem auditory-evoked responses associated with homozygous sickle cell disease

To determine the status of the cochlea, auditory pathway, and hearing threshold in patients with sickle cell disease, we recorded brainstem auditory-evoked potential (BSAEP) and performed electrocochleography (ECOG). This observational study was performed in the Department of Clinical Physiology at our hospital from January 2002 to November 2009. We studied patients diagnosed with sickle cell disease and having varying degrees of hearing impairment. BSAEP and ECOG data were recorded according to standard protocols. We observed 3 result categories. Group I (n=5) had a moderate increase in hearing threshold. Group II (n=8) at 80 dB had increased I-V interpeak latency and increased III-V interpeak latency with normal electrocochleographic data and a significantly high hearing threshold. In group III (n=1), only a delayed wave V was present at 80 dB and the electrocochlear response was absent. Most cases of sickle cell disease were associated with varying degrees of hearing impairment with increased hearing threshold and predominantly retrocochlear involvement.