Background: Mutations in GJB2 are the most common molecular defects responsible for autosomal recessive nonsyndromic hearing impairment (NSHI). The mutation spectra of this gene vary among different ethnic groups.

Parents were satisfied with all domains of HRQoL. Almost all domains exhibited rapid progress over the first 3 months of CI use, with education progressing at a slower rate. This research underscores the importance of language rehabilitation by revealing that strengthening language rehabilitation could be an effective means of improving the HRQoL of children with CIs.

The objective of this study is to make a clinical analysis for first branchial cleft anomalies (FBCAs), especially introduce the relationship between the Type I/II FBCA with the facial nerve and to demonstrate the importance of using intraoperative microscope and facial nerve monitoring. This is a retrospective review of patients with FBCAs treated in Beijing Children's Hospital, from Jan 2013 to Dec 2015. Clinical data of patients, including sex, age, chief complains, history of surgery, incision and drainage, the relationship with the facial nerve, pre and post-operative facial paralysis, recurrent rate and complications were recorded. FBCAs were divided into two subtypes according to Work's Classification. All patients had an MRI examination for diagnosis and to locate the lesions before surgery. Both microscope and facial nerve monitors were routinely used for detecting and protecting the facial nerve. The study cohort included 42 patients with ages ranging from 10 months to 14 years. The chief complaint was recurrent swelling or abscess near the ear or angle of mandibular. 76.2% of them (32/42) had incision and drainage histories. Two of them had suffered from facial palsy during the infectious period. Seven patients had undergone surgeries once or twice in other hospitals. Four patients with Type II FBCAs developed temporary facial palsy. We had follow-ups for each patient from 3 months to 3 years. 4 (9.5%) patients with Type I FBCA had got postoperative external auditory canal (EAC) stenosis. There were no cases of recurrence. Type II FBCAs has closer relationship with facial nerve than Type I FBCAs. The facial nerve of Type II FBCAs may lie more superficially and ascending to the fistula. Intraoperative microscope and facial nerve monitoring were necessary for protecting the facial nerve and decreasing the recurrent rate.