Indian Journal of Otology

ORIGINAL ARTICLE
Year
: 2019  |  Volume : 25  |  Issue : 4  |  Page : 188--191

Ear anomalies (congenital) at referral hospital


Vikas Sinha, Sushil Jha, Jigna Savani 
 Department of E.N.T., Sir. T. General Hospital, Government Medical College, Bhavnagar, Gujarat, India

Correspondence Address:
Dr. Vikas Sinha
Department of E.N.T., Sir. T. Hospital, Bhavnagar - 364 001, Gujarat
India

Abstract

Background: In otorhinolaryngology region, about half of the malformation affects the ear. Congenital anomalies of the ear can affect the external ear, middle ear, and inner ear. The ear malformations can be unilateral or bilateral. In this study, we enrolled patients with preauricular cyst, tag, accessory tragus, preauricular sinus, pinna sinus, microtia, anotia, EAC atresia, and congenital sensorineural hearing loss (SNHL). Hearing loss may lead to delayed language development and poor school performance. Universal newborn hearing screening allows for earlier detection and intervention in infants with hearing loss, which may result in improved language development in affected children. Estimated rates of congenital SNHL range from 1/1000 to 3/1000 live births. Aim and Objective: Proper and early diagnosis can change the future of a child by providing hearing rehabilitation and speech therapy. Materials and Methods: It is a prospective study of 122 patients of ear-related anomalies, conducted at the Government Medical College and Sir T. Hospital, Bhavnagar, Gujarat, India, from January 2016 to July 2018. Results: we have observed patients with otological malformations which includes external, middle and inner ear malformations. Conclusion: This study contains 122 patients, of which more than half of the patients are male (54.91%). We obsereved patients with Congenital SNHL, pinna anomalies, congenital facial weakness. We have also obtained cases with Waardenburg syndrome and Goldenhar syndrome.



How to cite this article:
Sinha V, Jha S, Savani J. Ear anomalies (congenital) at referral hospital.Indian J Otol 2019;25:188-191


How to cite this URL:
Sinha V, Jha S, Savani J. Ear anomalies (congenital) at referral hospital. Indian J Otol [serial online] 2019 [cited 2020 Feb 24 ];25:188-191
Available from: http://www.indianjotol.org/text.asp?2019/25/4/188/272223


Full Text



 Introduction



In otorhinolaryngology region, about half of the malformation affects the ear. Congenital anomalies of the ear can affect the external ear (pinna and external auditory canal [EAC]), middle ear, and inner ear. The ear malformations can be unilateral or bilateral. In this study, we enrolled patients with preauricular cyst, tag, accessory tragus, preauricular sinus, pinna sinus, microtia, anotia, EAC atresia, and congenital sensorineural hearing loss (SNHL). Hearing loss may lead to delayed language development and poor school performance. Universal newborn hearing screening allows for earlier detection and intervention in infants with hearing loss, which may result in improved language development in affected children. Estimated rates of congenital SNHL range from 1/1000 to 3/1000 live births. Proper and early diagnosis can change the future of a child by providing hearing rehabilitation and speech therapy.

The diagnostic steps include clinical examination, audiological testing, genetic analysis, and imaging modalities (computed tomography [CT] scan and magnetic resonance imaging (MRI) temporal bone). These imaging methods are most usefully employed in combination. Precise description of the malformations using CT and MRI is indispensable for the planning and successful outcome of operative ear reconstruction and rehabilitation procedures including cochlear implantation. A clinician must look for other systemic examination such as renal, ophthalmic, gastrointestinal, and cardiac anomalies.

 Methodology



It is a prospective study of 122 patients of ear-related anomalies, conducted at the Government Medical College and Sir T. Hospital, Bhavnagar, Gujarat, India, from January 2016 to July 2018. The study consists of patients presented to the Ear, Nose, and Throat Department, and pediatric department with the head-and-neck congenital anomalies. The patient examined thoroughly and evaluated for otorhinolaryngological and other systemic abnormalities. For ear-related developmental anomalies history includes decreased hearing, deformity of the external pinna or EAC, preauricular tags, preauricular sinus, and accessory tragus. The hearing assessment was done with startle reflex, tuning fork tests, free-field audiometry, otoacoustic emissions, brainstem evoked response audiometry (BERA) test, and the auditory steady-state response (ASSR) test. MRI temporal bone done to rule out cochlear malformation. Most newborns with hearing loss do not have obvious abnormalities on physical examination, but the ophthalmologic examination is warranted in these patients because of the high incidence of ocular defects in patients with severe to profound hearing loss.

 Observation and Discussion



In this study, we enrolled 122 cases, of which 67 (54.91%) were male and rest of 45 (36.89%) cases were female [Table 1].[1]{Table 1}

Pinna and external auditory canal anomalies

Total 30 (24.6%) patients have pinna abnormalities, of which 17 (56.7%) were male and rest 13 (43.3%) cases were female [Table 1].[1],[2],[3]

In this study, all cases enrolled with the bilateral presentation. Patients with anotia had conductive hearing loss; whereas four patients of microtia [Figure 1]a and [Figure 1]b were associated with SNHL. One male patient of bilateral microtia was associated with left-sided facial weakness.{Figure 1}

In this study, five (4.09%) patients of bilateral preauricular sinus [Figure 2] and [Figure 3] were recorded. Of which, two patients were male and three patients were female [Table 2].[4],[5]{Figure 2}{Figure 3}{Table 2}

The accessory tragus can be found on imaginary line drown from the tragus to angle of the mouth [Figure 4].[6] Five cases of the accessory tragus, eight cases of preauricular tag, and seven cases of preauricular cyst were identified [Table 3].{Figure 4}{Table 3}

A total of 63 (51.63%) patients were noted with congenital sensory hearing loss [Table 1].[1],[4] There were 37 (58.7%) male children and 26 (41.2%) female children. All affected bilaterally. Each patient was evaluated by audiological procedures such as tuning fork tests, pure-tone audiometry, free-field audiometry, BERA and ASSR, and MRI temporal bone in selective cases. The prelingual patients were encouraged for cochlear implant surgery and bone-anchored hearing aid. Three patients of Waardenburg syndrome and one case of Goldenhar syndrome were identified.[7],[8],[9]

Total four (3.20%) patients of congenital facial weakness were noted. Of which two (50%) neglected female cases of the right congenital facial weakness were notified at the age of 16 years and 33 years, whereas another two male children were identified with left-sided facial weakness at the age of 14 days and 5 years.[10]

 Conclusion



This study contains 122 patients, of which more than half of the patients are male (54.91%).

Congenital SNHL accounting for approximately half of the patients. Syndromic children with Waardenburg syndrome and Goldenhar syndrome were notified [Figure 5].{Figure 5}

There were 30 patients with pinna anomalies, with male predominance (17 cases). Microtia is the most common anomaly as far as pinna anomalies considered.

Of five patients of preauricular sinus, two were male and three were female. All cases were recorded with bilaterally and presented with local site infection.

Five cases of the accessory tragus, eight cases of preauricular tag, and seven cases of preauricular cyst were identified.

Four cases of congenital facial weakness were enrolled [Figure 6]. They have not a history of an obstructed labor.{Figure 6}

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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