Conductive hearing loss and otopathology in cleft palate patients

Conductive hearing loss and otopathology in cleft palate patients

Otolaryngology–Head and Neck Surgery (2006) 134, 946-948 ORIGINAL RESEARCH Conductive hearing loss and otopathology in cleft palate patients Steven ...

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Otolaryngology–Head and Neck Surgery (2006) 134, 946-948

ORIGINAL RESEARCH

Conductive hearing loss and otopathology in cleft palate patients Steven Goudy, MD, David Lott, MD, John Canady, MD, and Richard J.H. Smith, MD, Nashville, Tennessee; Cleveland, Ohio; and Iowa City, Iowa OBJECTIVES: Assess incidence of conductive hearing loss, ear pathology, and associated communicative disorders in cleft palate patients. STUDY DESIGN: Retrospective chart review of 101 patients all treated at a tertiary facility since birth. RESULTS: The median patient age was 19 years old (range 8-25) at last follow-up, 35% female. Median age of cleft palate repair was 16 months (range 12-60). Median number of myringotomy tubes was 3 (range 1-7). Conductive hearing loss (CHL) greater than 20 db PTA was found in 25% of patients at last follow-up. Severity of CHL was mild in 75%, moderate in 21%, and severe in 4%. Cholesteatoma was identified in 5.9%. The mean age at resolution of CHL was 5 years (range 3-19). Risk factors associated with CHL at last follow-up included middle ear surgery (P ⫽ 0.016), cholesteatoma (P ⫽ 0.003), and 4 or more myringotomy tube insertions (P ⫽ 0.030). Associations between CHL and age at cleft repair, speech impairment, or learning disabilities were not found. CONCLUSIONS: Children requiring increased number of myringotomy tubes and middle ear surgery and found to have cholesteatoma are at increased risk for long-standing CHL. EBM rating: C-4 SIGNIFICANCE: Cleft palate children requiring multiple tube insertions should be monitored closely for CHL. © 2006 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved.

function and increased eustachian tube compliance.3,4 Middle ear pathology leads to conductive hearing loss in 50% to 93% of patients.5,6 With the advent of myringotomy tube insertion, there were significant improvements in hearing.7 Upon repair of the cleft palate, 50% of patients will normalize their eustachian tube dysfunction (ETD).8,9 Resolution of ETD in the majority of patients normalizes by the time they are 5 or 6 years of age in 75% to 94% of patients.7,10,11 Long-term hearing loss with cleft palate patients occurs in 2% to 24%.7,12,13 This study examines what risk factors are associated with otopathology and long-term conductive hearing loss in cleft palate patients.

MATERIALS AND METHODS

he presence of middle ear effusions in children with cleft palate is almost universal.1,2 The incidence of clefting is 1:500-1000 live births, making it one of the most common congenital defects. The etiology of otitis media with effusion (OME) is due to poor tensor veli palitini

After receiving institutional review board approval, the charts of patients 8 years or older treated solely at the University of Iowa craniofacial clinic were reviewed. Routine evaluation of these patients included serial exams every 6 months with audiologic and physical exam. Note was made of type of cleft, age of palate repair, age of first tube insertion, total number of tube insertions, presence of tympanosclerosis or retraction pocket, ear surgery, cholesteatoma, speech surgery, and learning disabilities. Patients with syndromes or sensorineural hearing loss were excluded. Palate surgeries were all performed using the two-flap palatoplasty technique.

From the Department of Otolaryngology, Vanderbilt University, Nashville (Dr Goudy); Department of Otolaryngology, Cleveland Clinic (Dr Lott); and Department of Otolaryngology, University of Iowa Hospitals and Clinics (Drs Canady and Smith).

Reprint requests: Steven Goudy, Department of Otolaryngology, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 372328605. E-mail address: [email protected].

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0194-5998/$32.00 © 2006 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2005.12.020

Goudy et al

Conductive hearing loss and otopathology in cleft . . .

Audiologic data was analyzed by computing the pure tone average (PTA) threshold using 500, 1000, and 2000 Hz using Grason Stadler audiometers and Madsen Zodiac tympanometers. Abnormal air conduction was judged to be a PTA greater than 20 dB in either ear. Recovery of ETD was determined by normalization of previous conductive hearing loss greater than 20 dB. Data were analyzed using statistical analysis: Fischer’s exact test on demographic data and Wilcoxon rank-sum testing on audiologic data.

RESULTS One hundred one patients were identified from 1979 to 1996 in a sequential fashion who had adequate follow-up, which represented all the cleft patients seen during this time period. The median patient age was 19 years (range 8-25). There were 36 girls and 65 boys. Median age at palate repair was 16 months (range 12-60). Ninety percent of patients were Caucasian and the other 10% Hispanic and African-American. Ninety percent had a history of chronic otitis media with effusion (COME), whereas only 19% had a history of acute otitis media. Hearing aids were worn by 2%. Learning disabilities were identified in 8%. Surgery for velopharyngeal insufficiency was necessary in 25%. All cleft palate patients were initially treated with myringotomy and tubes, either at the time of lip repair or after persistent middle ear effusion (MEE) greater than 3 months. MEE requiring tympanostomy tube insertion was necessary a median of 3 times (range 1-7). Conductive hearing loss greater than 20 dB in either ear never occurred in 47.5% of patients, resolved in 27.7% of patients at last follow-up, and persisted in 24.8%. The median resolution of conductive hearing loss was 5 years (range 3-19). Cholesteatoma was identified in 5.9% of patients, and 12.9% required middle ear surgery. Middle ear surgeries included 2 myringoplasties, 8 tympanoplasties, 6 tympanomastoidectomies, 1 ossicular chain reconstruction, and 1 bone-anchored hearing aid placement. Factors associated with a conductive hearing loss at last follow-up were cholesteatoma (P ⫽ 0.003), middle ear surgery (P ⫽ 0.016), and patients with 4 or more tympanostomy tube insertions (P ⫽ 0.030). Of the patients with conductive hearing loss at last follow-up, 75% were mild (20-40 dB), 21% were moderate (41-60 dB), and 4% were severe (⬎60 dB). Of these losses, 44.4% were bilateral. There was no statistical correlation (P ⬎ 0.05) between hearing outcomes and gender, race, age at palate repair, history of acute or chronic otitis media, learning disability, tympanosclerosis, age at first tympanostomy tube insertion, or surgery for velopharyngeal insufficiency.

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DISCUSSION In our cleft palate patients we found a 91% rate of COME, which is comparable to earlier reports.1 Ventilation of the middle ear space for COME was standard treatment in this study. Routine use of myringotomy tubes has been cautioned by other authors due to presumed hearing loss from tube placement.14,15 The average patient underwent 3.1 myringotomy tube placements, and patients receiving 4 or more sets of tubes were at a greater risk of persistent conductive hearing loss. Sheahan et al (2002, 2003)13,16 also noted a similar propensity to hearing loss associated with 2 or 3 sets of tubes. The unanswered question is whether the hearing loss is due to myringotomy tube placement, suggesting a more conservative management of OME, or due to the damage to the middle ear from inflammation associated with OME. Pursuant to this, patients with cholesteatoma and those who underwent middle ear surgery also were at greater risk for conductive hearing loss. Again, is this related to tube insertion or chronic effects of OME in the middle ear? Previously reported incidence of cholesteatoma in cleft palate patients here in Iowa was 2.6%,17 which is higher than 0.9% found in Norway.12 Persistent conductive hearing loss in cleft palate patients is 24% in our study and is similarly reported in other studies,7,13 which is markedly different than 11% in Smith et al11 or 1% in Moller et al.12 The majority of the hearing loss was less than 40 dB, but it is important to note that 44% of patients had bilateral CHL. The discrepancy in hearing loss frequency may be related to fewer myringotomy tube insertions in Smith et al11 where T-tubes were used predominantly, or when tubes were infrequently used in Moeller et al.12 This is in contrast to the study by Tuncbilek et al18 of children with cleft palate who did not undergo myringotomy, where 37% had hearing loss greater than 15 dB. These differences may be related to tube type, number of tube insertions, epidemiology of OME in these countries, or the retrospective nature of all of these papers. Schonweiler et al19 found that children with conductive hearing loss who did not receive myringotomy tubes had significantly more speech-language problems, which eventually normalized with treatment. Our study did not address this, but it is important to bear in mind the impact of hearing loss, even if transient, in the development of speech and language in children already at a disadvantage. Children who receive myringotomy tubes early and have functioning tubes have been shown to have better hearing thresholds.6,20 The resolution of conductive hearing loss in our patients at the age of 5 years correlated well with other reports of ETD normalization.7,10,11 No correlation was identified between permanent conductive hearing loss and gender, race, age of cleft repair, time of first myringotomy tube insertion, history of acute or chronic otitis media, tympanosclerosis, learning disability, or surgical repair of velopharyngeal insufficiency. Sheahan et al16 did find a correlation between abnormal appearance

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Otolaryngology–Head and Neck Surgery, Vol 134, No 6, June 2006

of the tympanic membrane and hearing loss, where we did not find a correlation with tympanosclerosis. A limitation of this study is that higher-frequency hearing losses may have been unrecognized due to no highfrequency audiologic data on the majority of our patients.

CONCLUSION Otitis media with effusion occurs ubiquitously in cleft palate patients. Myringotomy tube insertion for treatment of otitis media with effusion and/or conductive hearing loss is common practice, although its usefulness is questioned. Eustachian tube dysfunction is largely responsible and resolves after palate repair in 50% or more, and resolves in most patients by the age of 5. Thought should be given to the use of long-term ventilation tubes in patients requiring multiple sets, due to the association of hearing loss with greater than 3 or 4 tube insertions. In patients with persistent conductive hearing loss, 44% were bilateral, warranting close observation and treatment. Lifelong otologic evaluation is recommended due to the 5.9% risk of cholesteatoma.

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6. Fria TJ, Paradise JL, Sabo DL, et al. Conductive hearing loss in infants and young children with cleft palate. J Pediatr 1987;111:84 –7. 7. Gould HJ. Hearing loss and cleft palate: The perspective of time. Cleft Palate J 1990;27:36 –9. 8. Bluestone CD, Wittel RA, Paradise JL. Roentgenographic evaluation of the Eustachian tube function in infants with cleft and normal palates. Cleft Palate J 1972;9:93–100. 9. Bellis ME, Passy V. Long-term hearing effects in cleft palate patients. Ear Nose Throat J 1987;66:49 –55. 10. Moller P. Hearing, middle ear pressure and otopathology in a cleft palate population. Acta Otolaryngol 1981;92:521– 8. 11. Smith TL, DiRuggiero DC, Jones KR. Recovery of Eustachian tube function and hearing outcome in patients with cleft palate. Otolaryngol Head Neck Surg 1994;111:423–9. 12. Moller P. Long-term otologic features of cleft palate patients. Arch Otolaryngol 1975;101:605–7. 13. Sheahan P, Miller I, Sheahan JN, et al. Incidence and outcome of middle ear disease in cleft lip and/or cleft palate. Int J Pediatr Otorhinolaryngol 2003;67:785–93. 14. Robson AK, Blanshard JD, Jones K, et al. A conservative approach to the management of otitis media with effusion in cleft palate children. J Laryngol Otol 1992;106:788 –92. 15. Ovesen T, Blegvad-Andersen O. Alterations in tympanic membrane appearance and middle ear function in 11-year-old children with complete unilateral cleft lip and palate compared with healthy age-matched subjects. Clin Otolaryngol 1992;17:203–7. 16. Sheahan P, Blayney AW, Sheahan JN, et al. Sequelae of otitis media with effusion among children with cleft lip and/or cleft palate. Clin Otolaryngol 2002;27:494 –500. 17. Dominguez S, Harker LA. Incidence of cholesteatoma with cleft palate. Ann Otol Rhinol Laryngol 1988;97:659 – 60. 18. Tuncbilek G, Ozgur F, Belgin E. Audiologic and tympanometric findings in children with cleft lip and palate. Cleft Palate Craniofac J 2003;40:304 –9. 19. Schonweiler R, Lisson JA, Schonweiler B, et al. A retrospective study of hearing, speech and language function in children with clefts following palatoplasty and veloplasty procedures at 18-24 months of age. Int J Pediatr Otorhinolaryngol 1999;50:205–17. 20. Broen PAA, Moller KT, Carlstrom J, et al. Comparison of the hearing histories of children with and without cleft palate. Cleft Palate Craniofac J 1996;33:127–33.