The aim of the present study was to compare the benefit of monaural versus binaural ear-level frequency modulated (FM) fitting on speech perception in noise in children with normal hearing. Reception threshold for sentences (RTS) was measured in no-FM, monaural FM, and binaural FM conditions in 22 normally developing children with bilateral normal hearing, aged 8 to 9 years old. Data were gathered using the Pediatric Malay Hearing in Noise Test (P-MyHINT) with speech presented from front and multi-talker babble presented from 90°, 180°, 270° azimuths in a sound treated booth. The results revealed that the use of either monaural or binaural ear level FM receivers provided significantly better mean RTSs than the no-FM condition (P<0.001). However, binaural FM did not produce a significantly greater benefit in mean RTS than monaural fitting. The benefit of binaural over monaural FM varies across individuals; while binaural fitting provided better RTSs in about 50% of study subjects, there were those in whom binaural fitting resulted in either deterioration or no additional improvement compared to monaural FM fitting. The present study suggests that the use of monaural ear-level FM receivers in children with normal hearing might provide similar benefit as binaural use. Individual subjects' variations of binaural FM benefit over monaural FM suggests that the decision to employ monaural or binaural fitting should be individualized. It should be noted however, that the current study recruits typically developing normal hearing children. Future studies involving normal hearing children with high risk of having difficulty listening in noise is indicated to see if similar findings are obtained.
Conductive hearing losses are typically present in disorders of the external/middle ear. However, there is a rare group of inner ear conditions called third windows that can also generate a conductive hearing loss. This is due to an abnormal connection between the middle and the inner ear or between the inner ear and the cranial cavity. X-linked gusher disorder is an extremely rare congenital inner ear dysplastic syndrome with an abnormal connection due to a characteristic incomplete cochlear partition type III and an incomplete internal auditory meatus fundus. The disorder is inherited in an X-linked fashion due to the mutation of the POU3F4 gene. We present two siblings diagnosed with the condition and their long-term follow-ups. They both presented audiovestibular symptoms and showed progressive mixed losses and bilateral vestibular weakness. They were treated with cochlear implant, digital amplification and with vestibular rehabilitation. Significant others around them were involved in their journey with the medical team, and in both, a very favourable outcome was achieved. This is the first time that we have reported evolving audiovestibular function with vestibular quantification in X-linked gusher disorder and emphasize on the multidisciplinary holistic approach to manage these children effectively.