Clinical diagnosis of Treacher Collins syndrome and the efficacy of using BAHA

Abstract

Objective:To evaluate the efficacy of soft or implanted BAHA in the patients of Treacher Collins syndrome(TCS).Method:Six patients of TCS were studied. The Teber scoring system was used to evaluate the deformity degree. The air and bone auditory thresholds were assessed by auditory brain stem response(ABR). The infant-toddler meaningful auditory integration scale(IT-MAIS) was used to assess the auditory development at three time levels: baseline,3 months and 6 months. The hearing threshold and speech recognition score were measured under unaided and aided conditions. Result:The average score of deformity degree was 14.0±0.6. The TCOF1 gene was tested in two patients. The bone conduction hearing thresholds of patients was（18.0±4.5）dBnHL and the air conduction hearing thresholds was （70.5±7.0）dBnHL. The IT-MAIS total, detection and perception scores were improved significantly after wearing softband BAHA and approached the normal level in the 2 patients under 2 years old. The hearing thresholds of 6 patients in unaided and softband BAHA conditions were（65.8±3.8）dBHL and （30.0±3.2）dBHL (P<0.01) respectively, and 1 implanted BAHA was 15 dBHL. The speech recognition scores of 3 patients in unaided and softband BAHA conditions were（31.7±3.5）% and（86.0±1.7）%(P<0.05) respectively, and 1 implanted BAHA was 96%. Conclusion:Whenever the patient was diagnosed as TCS by the clinical manifestations and genetic testing, BAHA system could help to rehabilitate the hearing to a normal condition.