An audiological perspective on ‘‘Two further patients with Warsaw breakage syndrome. Is a mild phenotype possible?

First of all, high‐resolution temporal bone CT revealed in both sisters the presence of cochlear malformation characterized by bilateral cystic aspect of the middle and apical turns consistent with incomplete partition type 2 without enlarged vestibular aqueduct (Sennaroğlu & Bajin, 2017). ( 2019) are not sufficient to explain the speech and language development delay, especially given the good auditory results at audiological testing by mean of play audiometry after sequential cochlear implantation (first cochlear implant performed at age 1.5 on the right side and second at 4 years old, aided PTA 32.5 dB nHL on the right and aided PTA 50 dB nHL on the left) that are appropriate for a normal speech and language development. [...]qualitative evaluation of ECAP responses, in terms of latency and shape of the action potential generated by the electric stimulation, although not conclusive, seemed to suggest that also auditory neuropathy can be excluded, at least for the intracochlear branch of the auditory nerve. [...]the cochlear nerves cannot be considered pathological and, at least, the peripheral electrical stimulation is efficient, while the speech discrimination may be not.