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Imaging Correlation of DFNB1 vs Non-DFNB1 Hearing Loss
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Otolaryngology-Head and Neck Surgery, Vol 139, No 2S1, August 2008
of the nose/midface (59%), followed by the mouth (51%), eye (48%), and forehead (35%). Overall inter-reader reliability was relatively strong for the midface (kappa⫽0.503) and global scores (kappa⫽0.541), followed by the mouth (k⫽0.419), the forehead (k⫽0.330), and the eye (k⫽0.302). There was a marked tendency for reader agreement to increase among those with more clinical experience. Reader agreement of the forehead (k⫽0.597) and eye (k⫽0.469) were strong only among experienced readers. CONCLUSIONS: In patients with differential facial function, a single global score is inadequate to describe facial function, and in this study, most directly correlated with the function of the midface. Regional assessment using the House-Brackmann grading scale more fully communicates facial function and increases in reliability with experience. Hearing Restoration with the RWI in Mixed Hearing Losses Vittorio Colletti, MD (presenter); Marco Carner, MD; Sheila Veronese; Liliana Colletti OBJECTIVE: The indications of the Vibrant Soundbridge (VSB), presently limited to the patients with sensorineural hearing loss (HL) and normal middle ear function, have been extended to include patients with mixed HL due to congenital or acquired ossicular chain defects. The FMT of the VSB placed onto the RW, round window implant (RWI), has allowed optimal amplification in patients with moderate-to-severe mixed HL who, at present, do not have good options for adequate functional rehabiliation. METHODS: 27 patients were unsuitable candidates for air and bone conductive hearing aids (BCHAs and ACHAs) and osseointegrative implants (BAHAs) and were treated with RWI. The patients were subdivided in 3 groups in relation to the etiologies and the performed surgeries. The first group includes 15 adults with unsuccessful OPLs (9 canal-wall-up (CWU) and 6 canal-wall-down (CWD) tympanoplasty); the second group includes 8 adult patients with CSOM, operated on dry radical or modified RCs; and the third group includes 4 patients, 2 children aged 1- and 10-years-old, and 2 adults with severe congenital malformations of the auricle combined with atresia of the outer ear canal and malformations of the ossicular chain. RWI surgeries will be illustrated in detail for each group. RESULTS: Significant improvements were observed in puretone threshold and speech understanding after surgery and at different follow-ups (12 -24 months), with no complications. CONCLUSIONS: The post-operative results suggest that RWI offers a viable treatment option for patients with severe mixed HL, regardless of the etiology of HL and previous surgeries.
Imaging Correlation of DFNB1 vs Non-DFNB1 Hearing Loss Amit Kochhar (presenter); Simon I Angeli, MD; Sandeep Dave, MD; Xue-Zhong Liu, MD, PhD OBJECTIVE: To evaluate temporal bone computerized tomography (CT) findings in children with DFNB1 hearing loss (HL) and non-DFNB1 deaf children, using absolute measurements and visual inspection. METHODS: A retrospective case-control series (1998 to 2005) was performed at an academic tertiary center. Children with non-syndromic HL were diagnosed as DFNB1 or nonDFNB1 after screening for GJB2 allele variants and the large GJB6 deletion. After matching for degree of HL, temporal bone CT images were compared in a cohort of 8 DFNB1 children (16 ears) to 9 non-DFNB1 children (18 ears). Visual criteria and absolute measurements were compared against normative values established by Purcell (2003). Absolute measurements between groups were compared using the student t-test. Non-parametric statistical tests were used when appropriate. Significance level was 0.05. RESULTS: Visual inspection failed to identify 2 patients with abnormalities found using absolute measurements. There was a statistically significant difference in the prevalence of abnormal temporal bone CT findings between DFNB1 (1 of 16 ears) and non-DFNB1 (10 of 18 ears) (p⬍0.0031, Fisher exact test). Of the absolute measurements, only the mean vestibule width in the non-DFNB1 group (4.195 ⫾ 0.5 mm) was significantly greater than in the DFNB1 group (3.65 ⫾ 0.2 mm) (p ⬍ 0.001). CONCLUSIONS: Visual inspection of temporal bone CT images alone may not adequately identify anomalies and should be used in conjunction with absolute CT measurements. Abnormal temporal bone CT findings are significantly less likely in children with DFNB1 when compared to non-DFNB1 children, despite matching for a similar degree of HL. Malleus Handle: Determinant of Success in Ossiculoplasty Anthony Bared, MD (presenter); Simon I Angeli, MD OBJECTIVE: Identify prognostic factors affecting outcome in ossicular chain reconstruction (OCR). METHODS: Retrospective case series of electronic database at an academic institution. We reviewed 209 cases of OCR performed from January 2000 through September 2007, and systematically collected demographic, clinical, audiologic, and outcome information. Univariate analyses of group differences in terms of postoperative air-bone gap (ABG) changes were evaluated by ANOVA. Multiple regression analyses were used to examine the relationship between postoperative air-bone gap (dependent variable) and the independent variables. RESULTS: There were 107 cases of OCR which met inclusion criteria (45 primary and 62 revision tympanoplasties) with
Otolaryngology-Head and Neck Surgery, Vol 139, No 2S1, August 2008
of the nose/midface (59%), followed by the mouth (51%), eye (48%), and forehead (35%). Overall inter-reader reliability was relatively strong for the midface (kappa⫽0.503) and global scores (kappa⫽0.541), followed by the mouth (k⫽0.419), the forehead (k⫽0.330), and the eye (k⫽0.302). There was a marked tendency for reader agreement to increase among those with more clinical experience. Reader agreement of the forehead (k⫽0.597) and eye (k⫽0.469) were strong only among experienced readers. CONCLUSIONS: In patients with differential facial function, a single global score is inadequate to describe facial function, and in this study, most directly correlated with the function of the midface. Regional assessment using the House-Brackmann grading scale more fully communicates facial function and increases in reliability with experience. Hearing Restoration with the RWI in Mixed Hearing Losses Vittorio Colletti, MD (presenter); Marco Carner, MD; Sheila Veronese; Liliana Colletti OBJECTIVE: The indications of the Vibrant Soundbridge (VSB), presently limited to the patients with sensorineural hearing loss (HL) and normal middle ear function, have been extended to include patients with mixed HL due to congenital or acquired ossicular chain defects. The FMT of the VSB placed onto the RW, round window implant (RWI), has allowed optimal amplification in patients with moderate-to-severe mixed HL who, at present, do not have good options for adequate functional rehabiliation. METHODS: 27 patients were unsuitable candidates for air and bone conductive hearing aids (BCHAs and ACHAs) and osseointegrative implants (BAHAs) and were treated with RWI. The patients were subdivided in 3 groups in relation to the etiologies and the performed surgeries. The first group includes 15 adults with unsuccessful OPLs (9 canal-wall-up (CWU) and 6 canal-wall-down (CWD) tympanoplasty); the second group includes 8 adult patients with CSOM, operated on dry radical or modified RCs; and the third group includes 4 patients, 2 children aged 1- and 10-years-old, and 2 adults with severe congenital malformations of the auricle combined with atresia of the outer ear canal and malformations of the ossicular chain. RWI surgeries will be illustrated in detail for each group. RESULTS: Significant improvements were observed in puretone threshold and speech understanding after surgery and at different follow-ups (12 -24 months), with no complications. CONCLUSIONS: The post-operative results suggest that RWI offers a viable treatment option for patients with severe mixed HL, regardless of the etiology of HL and previous surgeries.
Imaging Correlation of DFNB1 vs Non-DFNB1 Hearing Loss Amit Kochhar (presenter); Simon I Angeli, MD; Sandeep Dave, MD; Xue-Zhong Liu, MD, PhD OBJECTIVE: To evaluate temporal bone computerized tomography (CT) findings in children with DFNB1 hearing loss (HL) and non-DFNB1 deaf children, using absolute measurements and visual inspection. METHODS: A retrospective case-control series (1998 to 2005) was performed at an academic tertiary center. Children with non-syndromic HL were diagnosed as DFNB1 or nonDFNB1 after screening for GJB2 allele variants and the large GJB6 deletion. After matching for degree of HL, temporal bone CT images were compared in a cohort of 8 DFNB1 children (16 ears) to 9 non-DFNB1 children (18 ears). Visual criteria and absolute measurements were compared against normative values established by Purcell (2003). Absolute measurements between groups were compared using the student t-test. Non-parametric statistical tests were used when appropriate. Significance level was 0.05. RESULTS: Visual inspection failed to identify 2 patients with abnormalities found using absolute measurements. There was a statistically significant difference in the prevalence of abnormal temporal bone CT findings between DFNB1 (1 of 16 ears) and non-DFNB1 (10 of 18 ears) (p⬍0.0031, Fisher exact test). Of the absolute measurements, only the mean vestibule width in the non-DFNB1 group (4.195 ⫾ 0.5 mm) was significantly greater than in the DFNB1 group (3.65 ⫾ 0.2 mm) (p ⬍ 0.001). CONCLUSIONS: Visual inspection of temporal bone CT images alone may not adequately identify anomalies and should be used in conjunction with absolute CT measurements. Abnormal temporal bone CT findings are significantly less likely in children with DFNB1 when compared to non-DFNB1 children, despite matching for a similar degree of HL. Malleus Handle: Determinant of Success in Ossiculoplasty Anthony Bared, MD (presenter); Simon I Angeli, MD OBJECTIVE: Identify prognostic factors affecting outcome in ossicular chain reconstruction (OCR). METHODS: Retrospective case series of electronic database at an academic institution. We reviewed 209 cases of OCR performed from January 2000 through September 2007, and systematically collected demographic, clinical, audiologic, and outcome information. Univariate analyses of group differences in terms of postoperative air-bone gap (ABG) changes were evaluated by ANOVA. Multiple regression analyses were used to examine the relationship between postoperative air-bone gap (dependent variable) and the independent variables. RESULTS: There were 107 cases of OCR which met inclusion criteria (45 primary and 62 revision tympanoplasties) with