Evaluation of hearing thresholds in 3-month-old children with a cleft palate: the basis for a selective policy for ventilation tube insertion at time of palate repair

Hearing thresholds in children with a cleft palate prior to cleft palate repair are not widely documented, and audiological criteria for short-term ventilation tube insertion do not exist. The aims of this prospective study are to estimate hearing thresholds in 40 children with a cleft palate by 3-month developmental age with auditory brainstem responses (ABRs) under natural sleep and to estimate a hearing threshold guideline for short-term ventilation tube insertion. Our results show a wide range of air conduction hearing thresholds using click ABRs (2-4 Hz), which ranged from 25 to 102 dBnHL in the left ear and from 25 to 80 dBnHL in the right ear with means of 53 and 49 and standard deviations of 17 and 13 respectively. The bone conduction thresholds ranged from 0 to 55 dBnHL with a mean of 26 and a standard deviation of 13. Eighty-three per cent of children had flat, type B, on high-frequency tympanograms, indicative of middle ear effusion. Thirty per cent of the infants had a cleft palate associated with a known syndrome. Currently, it is the authors' practice to use short-term ventilation tubes on a selective basis at the time of cleft palate repair when there is a conductive hearing loss of more than 55 dBnHL in the better ear as determined by ABR with type B high-frequency tympanograms. This threshold level takes into account electrophysiological and auditory pathway maturation discrepancies. With this as the guideline, between 28% and 35% of the children in this study would be eligible for surgery. This criterion still requires further validation.     

腭裂患儿分泌性中耳炎鼓室置管术疗效分析

目的　观察鼓室置管术在治疗腭裂患儿分泌性中耳炎听力损失的疗效 ,探讨中耳通气管的选择、手术适应证及注意事项。方法　双耳伴发分泌性中耳炎伴听力损失的住院腭裂患儿 19例 ,平均年龄 5 8岁 ,平均气导语频听阈较大的一侧耳在腭裂修复术同期行鼓室置管术 ,对侧未置管耳作为对照组 ,术后 2周至 18个月复查 ,比较置管组及对照组腭裂术前、术后听阈的变化情况。结果置管组耳术后平均气导语频听阈 (2 7 0± 6 5 )dB较术前 (42 7± 8 2 )dB显著降低 ,而对照组耳术前(2 9 0± 6 1)dB、术后 (2 7 0± 4 1)dB听阈差异无显著性。置管组未见严重耳科并发症。结论　腭裂修复术同期鼓室置管术安全、有效 ,可恢复患者听力 ,有利于腭裂术后语音学习。     

Cleft palate and otitis media with effusion: a review

Otitis media with effusion is a common finding in children with cleft palate. However, although middle ear effusions have been reported to be almost universal among infants with cleft palate, less is known regarding the epidemiology, natural history, and outcome of middle ear disease among older children with cleft palate. Furthermore, considerable controversy surrounds the management of otitis media with effusion in these children: many authors have advocated a policy of early or scheduled ventilation tube insertion on account of the high incidence of hearing loss and speech difficulties; others have advocated a more conservative policy on account of the high incidence of complications from ventilation tubes and lack of evidence supporting a policy of early intervention. In the present paper, we review the epidemiology, aetiology, and natural history of middle ear disease in children with cleft palate. We also discuss the controversies involved in the management of these patients.     

腭裂与中耳功能障碍的相关性

目的：探讨腭裂手术对患者中耳功能的影响,为早期改善患者中耳功能提供依据。方法：对112例(224耳)腭裂患者进行年龄分组,采用声导抗、纯音测听或听性脑干反应检查。65例腭裂伴分泌性中耳炎患者在作腭裂修复术的同时,行鼓室置管术,术前术后作听力学检测,术前对鼓室分泌物作细菌培养。结果：腭裂患者常伴有不同程度的中耳功能障碍,腭裂修复术能明显改善患者中耳功能(P＜0.05)。鼓室分泌物Ｇ+、Ｇ-细菌培养阳性率为22.86%、23.53%,且多为低毒性条件致病菌。结论：腭裂患者常有中耳功能障碍,应重视其早期预防与治疗,早期修复腭裂有助于咽鼓管功能恢复。     

新生儿及婴儿期腭裂患儿分泌性中耳炎的发病情况

目的：了解新生儿及婴儿期腭裂患儿发生分泌性中耳炎（SOM）和听力损失情况,探讨其中耳功能障碍的发病时间规律及SOM早期预测方法。方法：出生后1～2周行听性脑干反应（ABR）筛查,无感音神经性聋的73例新生儿及婴儿腭裂患儿,在婴儿期于每月龄末行ABR和声导抗检查。结合鼓室导抗图形、静态声顺值、镫骨肌声反射和ABR波V阈值作为SOM的判断指标;以ABR波V阈值作为2～4kHz范围的听损伤分级标准。结果：73例（146耳）腭裂患儿发生疑似SOM,发生率为100％,均于出生后6个月内发生,平均发生年龄为2．5月龄。其中115耳（78．8％）1岁内发生SOM,平均发病年龄为5．4月龄,出生后6个月时为相对发病高峰期;SOM的前驱期平均为3．8个月。婴儿期腭裂患儿56．2％会出现不同程度的听力损伤,ABR波V阈值平均为48．6dBnHL。结论：婴儿期腭裂患儿SOM发病率高,可引起听力损失,严重影响中耳功能,其发生呈渐进性过程;作为发生中耳功能障碍的高危人群,出生后1个月时应行听力检查,每2～3个月定期复查,无条件者至少6～12个月进行耳科检查,以早期发现、及时诊断。     

学龄前腭裂患儿听力学特征分析

目的　对正常儿和腭裂患儿的听力学检查结果进行分析比对,以探讨腭裂患儿的听力学特点以及年龄因素的影响。方法　对60例（120耳）腭裂患儿和76例（152耳）正常儿进行听性脑干反应（auditory brainstem response,ABR）和声导抗检查。结果　≤1岁的腭裂患儿比1～2岁和2～6岁患儿的ABR阈值增加（前者S=5.927,后者S =6.020,P <0.05）。同一年龄段的患儿与正常儿相比,ABR阈值增加（t =8.588、5.436、7.003,P <0.001）,I、III、V波潜伏期明显延长（<1岁组t =9.388、4.727、2.398,1～2岁组t =5.386、6.218、5.255,2～7岁组t =7.038、7.282、7.308,P 均<0.05）。腭裂患儿的高频听力损失程度为轻、中度,无重度和极重度听力障碍。3个年龄段腭裂患儿阈值异常率、鼓室图异常率、镫骨肌声反射异常率和分泌性中耳炎患病率差异均无统计学意义（P >0.05）,而听力损失程度构成比差异有统计学意义（χ 2=14.20,P＜0.01）。结论　在学龄前婴幼儿腭裂群体中,当鼓室导抗图表现为B型或C型曲线,镫骨肌声反射未引出,同时ABR听阈增加,I、III、V波潜伏期延长,而各波间期无明显延长时,要高度警惕发生分泌性中耳炎,故应在早期行腭裂整复术同时进行鼓室置管术。     

The long‐term effect of unilateral t‐tube insertion in patients undergoing cleft palate repair: 20‐year follow‐up of a randomised controlled trial

Objectives: Between July 1984 and March 1987, all children that underwent repair for primary cleft palate at the Queen Victoria Hospital were enrolled in a clinical trial. Those found to have otitis media with effusion at time of surgery had a t‐tube inserted into one randomised ear, whilst the other ear received no treatment. The object of the study was to reassess the patients from the original trial to discover the impact of the unilateral t‐tube, twenty years later analysed on an intention to treat basis. Design: Children that underwent primary palatal closure during the study dates were identified from the cleft palate database. Patients that were involved in the original trial were identified, contacted and invited to take part in the follow‐up study. Setting: Tertiary referral Specialist unit. Participants: Twenty‐two patients were identified as potential study participants. Of this group, fourteen were contactable and seven participants agreed to take part in our follow‐up study. Main outcome measures: Persistent symptomatology, otoscopy, pure tone audiometry and tympanometry. Results: Follow‐up results were compared within the original treatment groups from the primary study, on an intention to treat basis. Otoscopically the ears were normal in 2 of the 7 treated ears compared with four of the seven non‐treated ears. All the other ear ears had various types of chronic otitis media. Four of the seven had hearing of >10 dB in the treated ear compared with the non‐treated ear. Conclusions: These findings would indicate need for caution in the use of t‐tubes in the cleft population and raises the question of long‐term follow‐up to assess for secondary cholesteatoma.     

A conservative approach to the management of otitis media with effusion in cleft palate children.

Otitis media with effusion (OME) is almost universal in children born with a cleft palate. Early placement of a ventilation tube to alleviate hearing problems is common. A retrospective study has been carried out to assess whether the practice of tube placement only for definite clinical indications is successful in terms of subsequent hearing levels and speech and language development. This was assessed by a case note review, analysis of speech therapy data and by means of a special follow-up clinic. There was no difference in speech development between those treated with tube insertion for OME and those untreated. Audiological thresholds were worse in the treated group. A similar number in each group required regular speech therapy. More abnormal otological findings were present at follow up in those who had tubes inserted, some of these were directly attributable to the presence of tubes. A conservative management of OME in cleft palate children, with tube insertion for only definite clinical indications, is an appropriate management, and will lead to fewer otological complications of tube insertion.     

腭裂修复术对患儿中耳功能的影响

目的 观察腭裂修复术对患儿中耳功能和听力的影响,为患儿听力问题的合理治疗提供依据.方法 197例患儿分为低年龄组（3～7岁）和高年龄组（8～12岁）.耳内镜下检查鼓膜像及咽鼓管咽口,鼓室图及纯音听阈测定.术后6个月复查.结果 患儿鼓膜的不可逆性病变8～12岁组明显高于3～7岁组.患儿咽鼓管咽口多呈裂隙状、圆脐状或过度宽大,咽鼓管咽口周围黏膜充血肿胀,有分泌物、食物残渣存留,术后得到明显改善.3～7岁组术后听力提高及A型鼓室图明显优于8～12岁组.结论 腭裂修复术后的软腭能分隔口腔和鼻腔,减少了鼻咽部感染的机会,有利于中耳功能恢复和听力的提高.提倡患儿早期手术,耳科医师应早期参与到治疗过程中,使患儿的中耳疾病在早期得到有效治疗.     

Incidence and outcome of middle ear disease in cleft lip and/or cleft palate

OBJECTIVE: Otitis media with effusion is known to be very common among children with cleft palate, however, less is known regarding the natural history and outcome in this group. The purpose of the present study was to examine the incidence, natural history, treatment, and outcome of middle ear disease in children with clefts. METHODS: A questionnaire was sent to the parents of all children registered on the cleft lip and palate database at our institution. The medical records of all respondents were also reviewed. Statistical analysis of the results was performed using Fisher's exact test in contingency tables and binary logistic regression analyses, where appropriate. RESULTS: 397 fully completed questionnaires were returned. Ear disease was much more common in children with cleft palate, or cleft lip and palate, than in children with cleft lip. Among children with cleft palate, ear problems (infections and/or hearing loss) were most prevalent in the 4-6-year-old age group. However, ear problems persisted at a substantial level for many years after this; only after the age of 12 years did problems appear to settle. The incidence of below normal current hearing and of surgery for chronic otitis media was significantly related to history of ear infections (P=0.000 and 0.000, respectively), and to increased number of ventilation tube insertions (P=0.000 and 0.000, respectively). CONCLUSIONS: Middle ear disease is common in children with cleft palate, and, unlike the case for children without clefts, has a prolonged recovery, and a substantial incidence of late sequelae. The higher incidence of below normal hearing and surgery for chronic otitis media in children undergoing a greater number of ventilation tube insertions, although most likely reflecting an increased underlying severity of otitis media in these children, also underlines the lack of long-term benefits of ventilation tubes in this group.     

Extended high-frequency hearing loss in children with cleft palate

Hearing sensitivity for frequencies 250 through 20 000 Hz was compared between children with repaired cleft palate and a history of otitis media, and children without cleft palate or middle ear disease. All subjects were clear of middle ear effusion during the evaluation. Although children in the cleft palate group had consistently poorer hearing throughout the auditory range, statistical analysis demonstrated significant differences in hearing levels above 9 000 Hz only. No statistical difference was observed for the standard clinical audiometric frequencies. Extra-high-frequency hearing loss appears to be associated with cleft palate and its sequelae.     

Sequelae of otitis media with effusion among children with cleft lip and/or cleft palate1

Otitis media with effusion (OME) is common among children with cleft palate, and may lead to such long‐term consequences as hearing loss, tympanic membrane retraction, and chronic otitis media (COM). In total, 104 children with cleft lip and/or palate treated for OME at our institution were reviewed. Mean duration of follow‐up was 6.9 years, and mean age at latest follow‐up was 9.6 years. The incidence of COM was 19%, and the incidence of cholesteatoma was 1.9%. Ears showing such long‐term sequelae of OME as hearing loss, tympanic membrane retraction, and chronic otitis media, were noted to have undergone a significantly greater number of ventilation tube insertions than ears not showing these sequalae. Our findings would suggest that a conservative approach to the management of OME in children with cleft palate is more likely to be beneficial in the long term.     

Parental perceptions of grommet insertion in children with cleft palate

Otitis media with effusion is almost universal in children with cleft palate and can delay speech, language and educational development by causing hearing loss. Grommet insertion at the time of cleft palate repair is common. There is debate about whether the benefits of grommets outweigh the risk of complications. A postal questionnaire was used to investigate parental perceptions of middle-ear ventilation via grommet insertion in children attending the multidisciplinary cleft palate clinic. These children's case notes were reviewed. Many children had speech and language delay, but parents thought this improved after grommet insertion. Overall parents were pleased with the results. This confirms that grommets have an important part to play in the management of children with cleft palate.     

Treatment of persistent middle ear effusion in cleft palate patients

Objectives

The goals of the research project are to learn how to individualize otologic care for cleft palate patients and to be able to counsel families of children with cleft palate on the benefit of tympanostomy tubes, hearing issues and risks of multiple sets of tubes.

Methods

The study is a retrospective chart review. Patients with a cleft palate with or without a cleft lip born between 1 January 2000 and 31 December 2005 referred to the Connecticut Children's Medical Center Craniofacial Department were included in the study. The patients were offered individualized ear surgery (PE tube placement) only if persistent middle ear fluid was present for over 3 months with a conductive hearing impairment. The primary outcome measures included the newborn hearing screening results, number of ear tube surgeries, and complications of PE tube insertion.

Results

There were 86 patients with cleft palate spectrum with or without cleft lip (45 females and 41 males). Twelve had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive. By 5 years old, 84 (98%) patients received at least one set of ear tubes for persistent middle ear fluid with conductive hearing impairment, while 2 received no tubes (2%). Of those who received ear tubes, the range was 1-6 with a mean of 1.7. Twelve patients (14%) had tympanosclerosis. Eight patients (9%) had eardrum perforation. One patient had myringoincudopexy. Of the 86 patients, 12 had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive.

Conclusions

(1) The majority of children born with cleft palate do not have middle ear fluid at birth. (2) Most children with cleft palate will likely develop persistent middle ear fluid with conductive hearing loss. Risks of complications from ear tubes in cleft palate patients are few and manageable using standard sized ear tubes.     

Otological findings in adults with isolated cleft palate or cleft lip, jaw, and palate]

BACKGROUND: Children with cleft palate often develop middle ear ventilation disorders due to chronic Eustachian tube dysfunction. This may lead to hearing loss. The insertion of ventilation tubes is a widely accepted measure to avoid sequelae of middle ear ventilation disorders and hearing loss. On the other hand, long-term therapy with ventilation tubes may inflict iatrogenic complications. The objective of the study was the evaluation of otoscopic and audiometric long-term findings in adult cleft patients who had been treated with ventilation tubes since childhood when chronic otitis media with effusion had been observed. PATIENTS AND METHODS: Ninety-two cleft palate patients had been followed up otoscopically and audiometrically for years. The average age was 19.3 years (minimum: 14, maximum: 39 years) at the time the last status was taken. RESULTS: Otoscopy revealed a perforation of the tympanic membrane in 3.8% of the 184 ears. 12% of the patients developed cholesteatoma, however three quarters of these occurred after age 11. 86.4% of the 92 patients had normal hearing in pure tone audiometry. CONCLUSION: Compared with adult cleft patients who did not receive ventilation tubes, our patients had a similar low incidence of eardrum perforations but a higher incidence of cholesteatomas while hearing loss occurred less often. Whether the higher incidence of cholesteatomas is caused iatrogenically or due to a longer follow-up period remains unclear. Whether the use of long-lasting ventilation tubes affects the incidence of cholesteatomas must be proved in further studies.     

Hearing levels and age in cleft palate patients

Tonal audiometry was used to compare hearing levels in patients with bilateral cleft lip and palate (BCLP) (57 patients), unilateral cleft lip and palate (UCLP) (124 patients) and isolated cleft palate (ICP) (62 patients), and according to age groups. Patients with isolated cleft palate showed greater improvement in hearing level with age than patients with UCLP and BCLP; as adults they showed the lowest frequency of ears with hearing level of less than 40 dB, and the highest frequency of ears with hearing levels of 11–20 dB. Patients with BCLP had a higher frequency of ears with a hearing level of 21–40 dB during early childhood and adult age than patients with ICP. Patients with UCLP and BCLP showed a slower decrease with age in the frequency of ears with hearing loss than patients with ICP; the hearing level in patients with UCLP and BCLP improved only in groups with hearing levels of 21–40 dB, while those with hearing levels above 40 dB showed no significant improvement with age.Conservative therapeutic methods for normalization of the hearing level and middle ear state were found to be inefficient in the cleft palate population Thus, early surgical treatment (ventilation tube insertion) appears to be the therapy of choice.     

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

Forty-four 11-year-old children born with a complete unilateral cleft lip and palate were examined to determine the frequency and the extent of changes in the tympanic membrane and the middle ear function and compared with 16 healthy children of the same age. The incidence of hearing impairment, abnormal middle ear pressure, retraction of the pars flaccida and abnormal tympanic membrane appearance were 24, 44, 23 and 67% respectively among the patients while the same parameters in the control group were 0, 12.5, 6 and 12%. Previous grommet insertion in the patient group was statistically correlated to both tympanic membrane abnormality and abnormal middle ear pessure but, remarkably, no association was found between grommet insertion and hearing impairment. The poor middle ear function in the children with cleft lip and palate was probably a result of reduced Eustachian tube function.     

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.

Forty-four 11-year-old children born with a complete unilateral cleft lip and palate were examined to determine the frequency and the extent of changes in the tympanic membrane and the middle ear function and compared with 16 healthy children of the same age. The incidence of hearing impairment, abnormal middle ear pressure, retraction of the pars flaccida and abnormal tympanic membrane appearance were 24, 44, 23 and 67% respectively among the patients while the same parameters in the control group were 0, 12.5, 6 and 12%. Previous grommet insertion in the patient group was statistically correlated to both tympanic membrane abnormality and abnormal middle ear pressure but, remarkably, no association was found between grommet insertion and hearing impairment. The poor middle ear function in the children with cleft lip and palate was probably a result of reduced Eustachian tube function.     

腭裂与分泌性中耳炎

目的探讨腭裂与分泌性中耳炎的关系,了解腭裂修复术及鼓室置管术手术前后的听力学变化.方法对203(406耳)例腭裂患者进行年龄分组,采用声导抗,纯音测听或听性脑干反应(ABR)检查判断中耳功能.21例腭裂伴分泌性中耳炎患者在作腭裂修复术的同时,行鼓室置管术,术前术后作听力学检测.结果203例腭裂患者,中耳功能障碍的发病率为68.5%,6岁前可高达80%.21例腭裂伴分泌性中耳炎患者术后随访1～3年,听力明显改善,平均听力比术前提高17dB(P＜0.01).结论腭裂与分泌性中耳炎的发病明显有关,年龄越小,发病率越高.腭裂伴有分泌性中耳炎的患者,应早期行鼓室置管术,方能改善听力,有助于正常语言的发育.     

畸变产物耳声发射在唇腭裂婴幼儿听力检查中的应用

目的探讨唇腭裂婴幼儿畸变产物耳声发射（distortion product otoacoustic emission,DPOAE）的特点,并将其与听性脑干反应（auditory brainstem response,ABR）阂值检查的结果加以对照,以探讨DPOAE在这类患儿听力检查中的应用价值。方法DPOAE检查63例（126耳）,年龄2个月-42个月,平均11.83个月。其中单纯腭裂组（以下简称腭裂组）23例（46耳）,腭裂并发唇裂及牙槽裂组（以下简称唇腭裂组）30例（60耳）,单纯唇裂组（以下简称唇裂组）10例（20耳）,每耳均检查8个频率,若2-5kHz4个频点有≥3个频点通过即为该耳通过。在上述患儿中,ABR阈值检查腭裂组17例（34耳）,唇腭裂组10例（20耳）,唇裂组6例（12耳）,以能重复引出V波的最小刺激强度为ABR阈值。结果DPOAE检查：腭裂组通过7耳,未通过39耳,通过率为15.22％;唇腭裂组通过6耳,未通过54耳,通过率为10.00％;唇裂组通过18耳,未通过2耳,通过率为90％。统计分析腭裂组与唇腭裂组无显著差异,而腭裂组与唇裂组,唇腭裂组与唇裂组均有显著差异。ABR阈值检查统计分析结果与DPOAE一致。将各组DPOAE通过率与ABR正常率进行比较,腭裂组及唇腭裂组中二者无差异（P〉0.05）,虽然唇裂组中二者有差异（P〈0.05）,但唇裂组ABR阈值反应的听力下降较轻（均≤50dB nHL）。从总体趋势上说,DPOAE与ABR阈值检查在检测的结果上是一致的。结论唇腭裂患儿DPOAE和ABR检测结果一致,与ABR相比,DPOAE具有快速、简便、易实施等特点,因此DPOAE可以作为唇腭裂婴幼儿听力检查的手段,但仍需进一步结合ABR及其他相关的听力检查,以明确听力损害的程度和类型。