小孩有時候同時存在聽力喪失與眼睛異常

湯包

作者：Laurie Barclay, MD  
出處：WebMD醫學新聞

　　February 16, 2009 —根據二月號頭頸外科和耳鼻喉期刊中發表的一篇回溯分析結果，感覺神經性聽力喪失(sensorineural hearing loss，SNHL)的小孩可能也會有眼睛異常。
　　
　　華盛頓大學的Arun Sharma醫師等人寫道，特別是在童年時，SNHL與語言、說話、認知及社會發展的延遲有關；因為聽力不佳，SNHL的小孩特別依賴其他獲得訊息的方法，如果這些小孩有未確認之會影響視力的眼科異常，對其發展可能會有更不利的結果。
　　
　　研究目標是檢視SNHL小孩的折射與非折射眼科異常，評估常規眼科檢查對SNHL小孩的整體診斷價值。
　　
　　研究者回顧226名18歲以下SNHL孩童，於2000年11月6日至2007年6月4日間，在一所四級大學兒童醫院眼科的檢查資料。
　　
　　有49名(21.7%)病患出現眼科異常，包括23名(10.2%)病患的折射異常以及29名(12.8%)病患的非折射異常。11名(4.9%)SNHL病患中，5人(2.2%)有與眼科異常相關的症狀。SNHL的嚴重度或單側性不會影響眼科的發現，新生兒篩檢時診斷者與其他病患之間的眼科發現流行率也沒有顯著不同。
　　
　　小孩半數的SNHL案例是遺傳所致，大多數白人病患的感覺神經性聽力喪失是因為GJB2基因，研究者因此也探究基因與眼科異常之間的關係，27名雙等位基因GJB2 突變病患有1人(3.7%) 、106名無任何GJB2 突變病患有22人 (20.7%)(P = .04)有眼科異常。
　　
　　研究作者寫道，這和GJB2 突變會導致SNHL但不會有其他異常或症狀的觀念一致；在本研究中，SNHL小孩之眼科發現的整體流行率為21.7%；GJB2 突變小孩有3.7%有眼科異常。
　　
　　研究限制包括這是回溯設計研究，對於所有SNHL小孩的眼科評估僅為建議而未全面進行，進行之眼科檢查的差異性。
　　
　　研究作者結論表示，多元方法對於評估與治療SNHL小孩是重要的，以確保符合他們的醫療、教育與社會需求。眼科評估有利於病患接受眼科醫師診斷（甚或治療）會影響視力的異常、以及幫助耳鼻喉科醫師確認SNHL的原因。
　　
　　作者宣稱沒有相關財務關係。

Hearing Loss and Eye Disorders Sometimes Coexist in Children

By Laurie Barclay, MD
Medscape Medical News

February 16, 2009 — Children with sensorineural hearing loss (SNHL) may also have eye disorders, according to the results of a retrospective analysis reported in the February issue of Archives of Otolaryngology and Head and Neck Surgery.

"Especially early in life, [SNHL] is associated with delays in language, speech, cognitive and social development," write Arun Sharma, MD, from the University of Washington, Seattle, and colleagues. "Given the effects of hearing impairment, children with [SNHL] are particularly dependent on other means of information acquisition. If these children were to have unrecognized ophthalmologic abnormalities that limited visual acuity, there could be further detrimental effects on development."

The goal of this study was to examine the prevalence of refractive and nonrefractive ophthalmologic abnormalities in children with SNHL and to assess the overall diagnostic value of routine ophthalmological examination in children with SNHL.

The investigators reviewed ophthalmologic findings from 226 children aged 18 years and younger with SNHL who underwent ophthalmologic evaluation at a tertiary care university children's hospital between November 6, 2000, and June 4, 2007.

Ophthalmologic abnormality was present in 49 patients (21.7%), including refractive errors in 23 patients (10.2%) and nonrefractive conditions in 29 patients (12.8%). Of 11 patients (4.9%) with SNHL, 5 (2.2%) had syndromes with associated ophthalmologic abnormalities. Severity or laterality of SNHL did not affect ophthalmologic findings, nor was the prevalence of ophthalmologic findings significantly different between patients diagnosed at newborn screening compared with other patients.

In children, half of all cases of SNHL result from genetic causes, with the GJB2 gene accounting for a large proportion of sensorineural hearing loss cases in white patients. The investigators therefore also looked at genetic associations with ophthalmologic abnormalities, which were present in 1 (3.7%) of 27 patients with biallelic GJB2 mutations and 22 (20.7%) of 106 patients without any GJB2 mutations (P = .04).

"This is consistent with the impression that GJB2 mutations result in [SNHL] but not in additional anomalies or syndromes," the study authors write. "In this study, the overall prevalence of ophthalmologic findings in children with SNHL was 21.7%. Ophthalmologic anomalies were present in 3.7% of children with GJB2 mutations."

Limitations of this study include its retrospective design, that ophthalmologic evaluation was recommended but not performed for all children with SNHL seen in the clinics, and variability in the ophthalmologic tests performed.

"A multidisciplinary approach is important in the evaluation and treatment of children with [SNHL] to ensure that their medical, education and social needs are met," the study authors conclude. "Ophthalmologic evaluation can be beneficial for patients by allowing ophthalmologists to diagnose (and possibly treat) co-existing disorders that affect vision and by helping otolaryngologists to determine the cause of [SNHL]."

The authors have disclosed no relevant financial relationships.

Arch Otolaryngol Head Neck Surg. 2009;135:119–123.