Figure 2From: Information from cochlear potentials and genetic mutations helps localize the lesion site in auditory neuropathyCochlear potentials recorded from patients with mutations in the OTOF or OPA1 gene. The upper panels show recordings from two subjects carrying, respectively, the R445 H mutation in OPA1 and biallelic mutations in OTOF. The recordings (black line) are superimposed on the mean summation potential-compound action potential (SP-CAP) response (gray line) from 16 controls with normal hearing at 120 dB sound pressure level intensity. The shaded area indicates the 95% confidence limit. The CAP response observed in controls is replaced in AN patients by a low-amplitude prolonged negative deflection following the SP. A small CAP (arrowed) is superimposed on the sustained response only in the child carrying biallelic OTOF mutation. The lower panels show the electrically evoked neural potentials (e-CAP) recorded through cochlear implant. A neural response was obtained only from patients with OTOF mutations.Back to article page