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

Ear anomalies (congenital) at referral hospital


Department of E.N.T., Sir. T. General Hospital, Government Medical College, Bhavnagar, Gujarat, India

Date of Submission21-Dec-2018
Date of Acceptance26-Mar-2019
Date of Web Publication4-Dec-2019

Correspondence Address:
Dr. Vikas Sinha
Department of E.N.T., Sir. T. Hospital, Bhavnagar - 364 001, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_123_18

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  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.

Keywords: Ear malformations, external auditory canal, hearing rehabilitation, pinna, sensorineural hearing loss, speech therapy


How to cite this article:
Sinha V, Jha S, Savani J. Ear anomalies (congenital) at referral hospital. Indian J Otol 2019;25:188-91

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 May 29];25:188-91. Available from: http://www.indianjotol.org/text.asp?2019/25/4/188/272223




  Introduction Top


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 Top


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 Top


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: Gender distribution in ear anomalies

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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: (a) Left ear microtia, (b) right ear microtia with canal atresia, (c) right ear anotia with canal atresia, (d) left ear anotia with canal atresia, (e) bilateral bat ear, (f) bilateral Darwin's tubercle

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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: (a) Right ear pinna preauricular sinus, (b) left ear pinna preauricular sinus

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Figure 3: Left pinna preauricular sinus

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Table 2: Gender distribution in pinna anomalies

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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: (a) Left ear preauricular tag, (b) right ear preauricular tag, (c) right ear preauricular cyst

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Table 3: Gender and side distribution in preauricular appendages

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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 Top


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: (a) A girl with left eye heterochromia and bilateral sensorineural hearing loss (Waardenburg syndrome), (b) bilateral isochromia with bilateral sensorineural hearing loss (Waardenburg syndrome), (c) left eye limbul dermoid with bilateral sensorineural hearing loss (Goldenhar syndrome)

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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: Left-sided congenital facial weakness

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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.



 
  References Top

1.
Sinha V, Johri S, George A. Congenital anomalies in E.N.T. Indian J Otolaryngol Head Neck Surg 2001;53:326-7.  Back to cited text no. 1
    
2.
Melnick M, Myrianthopoulos NC, Paul NW. External ear malformations: Epidemiology, genetics, and natural history. Birth Defects Orig Artic Ser 1979;15:i-ix, 1-140.  Back to cited text no. 2
    
3.
Lambert PR. Major congenital ear malformations: Surgical management and results. Ann Otol Rhinol Laryngol 1988;97:641-9.  Back to cited text no. 3
    
4.
Adegbiji WA, Alabi BS, Olajuyin OA, Nwawolo CC. Presentation of preauricular sinus and preauricular sinus abscess in Southwest Nigeria. Int J Biomed Sci 2013;9:260-3.  Back to cited text no. 4
    
5.
Tang IP, Shashinder S, Kuljit S, Gopala KG. Outcome of patients presenting with preauricular sinus in a tertiary centre – A five year experience. Med J Malaysia 2007;62:53-5.  Back to cited text no. 5
    
6.
Firat Y, Sireci S, Yakinci C, Akarçay M, Karakaş HM, Firat AK, et al. Isolated preauricular pits and tags: Is it necessary to investigate renal abnormalities and hearing impairment? Eur Arch Otorhinolaryngol 2008;265:1057-60.  Back to cited text no. 6
    
7.
Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL. Language of early- and later-identified children with hearing loss. Pediatrics 1998;102:1161-71.  Back to cited text no. 7
    
8.
Migirov L, Henkin Y, Hildesheimer M, Muchnik C, Kronenberg J. Cochlear implantation in Waardenburg's syndrome. Acta Otolaryngol 2005;125:713-7.  Back to cited text no. 8
    
9.
Digilio MC, Calzolari F, Capolino R, Toscano A, Sarkozy A, de Zorzi A, et al. Congenital heart defects in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome). Am J Med Genet A 2008;146A: 1815-9.  Back to cited text no. 9
    
10.
Falco NA, Eriksson E. Facial nerve palsy in the newborn: Incidence and outcome. Plast Reconstr Surg 1990;85:1-4.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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