Hearing improvement represents one of the may valuable outcomes in microtia and aural atresia reconstruction surgery. Most patients with poor development in their hearing function have had a severe microtia. Conventional methods to improve hearing function are bone conduction and bone anchored hearing aids. Cartilage conduction hearing aids (CCHA) represents a new amplification method. This study assessed the outcomes and evaluated the impact and its safety in the patients with microtia and aural atresia whose hearing dysfunction did not improve after surgery for ear reconstruction in our hospital. Hearing functions were evaluated with pure tone audiometry or sound field testing by behavioral audiometry and speech audiometry before and after CCHA fitting. As a result, there was a significant difference between unaided and aided thresholds (p < 0.001). Speech recognition threshold and speech discrimination level also significantly improved with CCHA. The average functional gains of 14 ears were 26.9 ± 2.3 dB. Almost all parents of the patients reported satisfaction with the performance of CCHA, and daily communication in children with hearing loss also became better than usual.

Latar belakang: Penyakit Mukopolisakaridosis (MPS) tipe II atau sindrom Hunter adalah gangguan terkait dengan kromosom X resesif yang disebabkan defisiensi enzim iduronate-2-sulfatase sehingga memicu penimbunan heparan sulfat dan dermatan sulfat. Gangguan pada pendengaran dan pernapasan bagian atas merupakan masalah umum yang terjadi pada penyakit ini. Tujuan: Untuk memberikan informasi mengenai gangguan pendengaran dan gangguan pernapasan bagian atas pada penyakit MPS tipe II. Kasus: Tiga pasien terdiagnosis MPS tipe II dengan gangguan pendengaran dan hipertrofi adenoid. Metode: Didapatkan 4 jurnal yang relevan setelah dilakukan penelusuran literatur dan penapisan dengan kriteria inklusi dan eksklusi. Hasil: Empat jurnal yang terdiri dari 3 laporan kasus dan 1 artikel penelitian menggambarkan gangguan pendengaran dan gangguan jalan napas bagian atas pada pasien dengan mukopolisakaridosis tipe II. Kesimpulan: Pendekatan multidisipliner diperlukan untuk mendiagnosis serta mengobati pasien MPS tipe II secara dini, agar dapat menunjang perkembangan individu dan sosial serta meningkatkan kualitas hidup pasien. Background: Mucopolysaccharidoses (MPS) type II is X-linked recessive disorder caused by iduronate-2-sulfatase enzyme deficiency and leads to the accumulation of heparan sulfate and dermatan sulfate. Hearing loss and upper respiratory track problems are commonlyoccur in this disorder. Purpose:To provide information about hearing loss and upper airway problems in MPS type II disorder. Cases: Three patients had been diagnosed as mucopolysaccharidoses type II with hearing loss and adenoid hypertrophy.Methods: Four relevant journals were obtained after literature searching and screening was performed using inclusion and exclusion criteria. Results: Out of 4 journals, 3 case reports and 1 research article were describing hearing loss and upper respiratory problems in patients with MPS type II. Conclusion:Multidisciplinary approach is needed for making early diagnosis and determining the treatment of mucopolysaccharidoses type II patients, in order to support individual and social development and to improve the patients’quality of life.

Latar belakang: Celah bibir dan palatum merupakan kelainan proses pertumbuhan area orofasial yang menimbulkan permasalahan kompleks. Penelitian ini menitikberatkan permasalahanyang terkait dengan fungsi bicara. Tujuan: Penelitian ini melaporkan gambaran skor nasalance padacelah palatum, hubungan antara skor nasalance percontoh celah palatum dan tanpa celah palatum sertafaktor-faktor yang berhubungan dengan skor nasalance. Metode: Desain penelitian adalah comparativecross sectional. Pengambilan percontoh dilakukan dengan purposive sampling. Dilakukan wawancara,pengisian kuesioner, pemeriksaan THT, audiometri, timpanometri, nasoendoskopi, dan nasometri.Hasil: Didapatkan gambaran rerata skor nasalance percontoh celah palatum. Terdapat  perbedaanbermakna antara skor nasalance percontoh celah palatum dan tanpa celah palatum pada Uji Gajah danUji Hantu (p<0,001). Pada analisis multivariat secara keseluruhan faktor-faktor yang berhubungandengan skor nasalance (adenoid, otitis media efusi, serta gangguan pendengaran) dan keberadaancelah palatum berpengaruh secara signifikan terhadap skor nasalance untuk semua uji nasalance(p<0,05) pada pengujian terhadap kedua kelompok percontoh. Bila dilihat secara parsial faktor adenoidberpengaruh secara signifikan terhadap skor semua uji nasalance baik pada analisis kedua kelompokpercontoh maupun pada percontoh celah palatum saja. Kesimpulan: Rerata skor nasalance kelompokcelah palatum lebih tinggi daripada kelompok tanpa celah palatum. Didapatkan peran faktor yangberpengaruh terhadap skor nasalance, khususnya adenoid. Diperlukan penelitian lebih lanjut terhadappercontoh sesudah operasi sehingga bisa dijadikan sebagai evaluasi terhadap keberhasilan tindakan. Kata kunci: celah palatum, skor nasalance, tonsil, adenoid, otitis media efusi, gangguan pendengaran ABSTRACTBackground: Cleft lip and palate is an orofacial malformation and associated with many problems. This study concerned in speech problem in cleft palate patients. Purpose: This study reportednasalance score in cleft palate patients, the correlation between nasalance score in cleft palate and noncleft palate groups and also some factors related with nasalance score in cleft palate patients. Methods:The design of this study was comparative cross sectional, with purposive sampling. Data was collectedwith interview, questioner apllication, ENT examination, audiometry, tympanometry, nasoendoscopy,and nasometry. Results: The result of this study reported the mean of nasalance score in cleft palatepatients. There was significant difference in nasalance score between cleft palate and non cleft palatepatients in Uji Gajah and Uji Hantu (p>0,05). In multivariate analysis, in general the factors relatedwith nasalance score (adenoid, otitis media with effusion, and hearing loss) and cleft palate  itself weresignificantly correlated with nasalance score in all nasalance test (p<0,05) in both groups analysis butno signifficant correlation in cleft palate group analysis. Partially, adenoid  was significantly correlated in both group analysis and cleft palate group analysis. Conclusion: The mean of nasalance score incleft palate group was higher than non cleft group. Some factors in this study, especially adenoid had corelations with nasalance score. It needs further study to evaluate the nasalance score patients withcleft palate after surgery to compare the results. Keywords: cleft palate, nasalance score, tonsil, adenoid, otitis media with effusion, hearing disturbance