ARCHIVED

Medical Policy

Policy Num:       07.001.033
Policy Name:     Otoplasty
Policy ID:          [07.001.033]  [Ac / L / M + / P+ ]     [0.00.00]

Last Review:       November 9, 2022
Next Review:      ARCHIVED
 

Related Policies:

None

OTOPLASTY

Summary

Reconstructive surgery of the ear can be considered for injury, trauma or congenital conditions. Congenital anomalies result from errors in embryogenesis (malformations) or

intrauterine events that affect embryonic and fetal growth (deformations and disruptions) [1]. The more complex the formation of a structure, the more opportunities for

malformation.

Objective

The objective of this evidence review is to determine whether repair or reconstruction of structures of the ear result in overall improvement in the health outcomes.

Policy Statements

Reconstructive surgery of the ear is considered for payment if it meets the following criteria:

• When the deformity or absence of the ear is the result of trauma or illness.

• If because of this condition there is significant loss of hearing. This must be documented.

It is not considered for payment if:

• This is for correcting large or prominent ears or injury resulting from piercing or from normal aging.

• The deformity is not for the reasons stated above.

Policy Guidelines

Cosmetic repair or reconstruction of ear structures secondary to piercings or aging are not considered as medically necessary.

For suture of wound or injury of external ear CPT codes 12011-14302.

For reconstruction of external auditory canal use CPT codes 69310-69320.

Benefit Application

BlueCard/National Account Issues

Not applicable

Background

Congenital outer ear anomalies include microtia, lop (outstanding, protruding) ear, preauricular pits, accessory auricular appendages/preauricular tags, and aural/external auditory canal atresia.

●Congenital middle ear anomalies include malformations of the ossicles (eg, fixation or incudostapedial discontinuity) and may result in conductive hearing loss. Ossicular malformations may occur as part of a syndrome or in isolation.

●Congenital inner ear anomalies include the Michel and Mondini malformations, and the enlarged vestibular aqueduct syndrome.

●External ear malformations and renal anomalies occur in several multiple congenital anomaly syndromes. We suggest that renal ultrasonography be performed in children with ear anomaly and any of the following:

•Other malformations or dysmorphic features

•Family history of deafness or auricular or renal malformations

•Maternal history of gestational diabetes

Regulatory Status

Not applicable

Rationale

Population Reference No. 1 Policy Statement

For individuals with congenital malformation causing significant incapacity. Interventions of interest are reconstruction of anatomical defect. Comparators of interest are reconstructive surgery. Relevant outcomes include overall quality of life, functional outcomes and symptoms.

OUTER EAR MALFORMATIONS

Microtia — Incomplete development and growth of the pinna can lead to a small or deformed pinna (microtia) or absent pinna (anotia).

●Definition and epidemiology – In unilateral cases, microtia is defined as a size discrepancy between ears that exceeds normal variation (ie, >10 percent difference) [10]. The right side is affected more commonly than the left. Bilateral microtia is defined as a length of the external ear that is more than 2 standard deviations below the mean [11]. In severe cases, the pinna is completely absent (anotia). Microtia and anotia occur in 1 to 3 per 10,000 births [12].

●Association with genetic syndromes – Microtia and anotia may occur in isolation or in association with genetic syndromes and other malformations, including facial clefts, cardiac defects, limb anomalies, renal anomalies, and holoprosencephaly [12,13]. Microtia is a common finding in children with Treacher-Collins syndrome and craniofacial microsomia (also called Goldenhar syndrome, hemifacial microsomia, oculo-auriculo-vertebral spectrum, and first and second branchial arch syndrome). (See "Syndromes with craniofacial abnormalities", section on 'Treacher Collins syndrome' and "Syndromes with craniofacial abnormalities", section on 'Craniofacial microsomia'.)

Numerous other microtia-associated syndromes have been reported (table 1); these may be associated with single-gene defects or chromosomal abnormalities [14]. Genetic factors also appear to play an important role in isolated, nonsyndromic cases [15]. In a series of 145 cases, more than two-thirds were sporadic and approximately one-third were familial [13]. Among the familial cases, autosomal dominant inheritance was most common, but cases with autosomal recessive and multifactorial inheritance were also noted.

●Other risk factors – Microtia and anotia occur more frequently in boys, at increased altitude, with increasing birth order, in infants of diabetic mothers, and infants with prenatal exposure to isotretinoin, thalidomide, alcohol, or mycophenolate [12,16-19].

●Classification – Several different classification systems for microtia have been proposed [14]. In the Marx system, type I microtia is characterized as a generally well-formed, perceptibly smaller auricle. In type II microtia, the pinna is malformed and 50 to 66 percent smaller than the contralateral pinna. In grade III microtia, the auricle is severely malformed and usually exhibits a peanut shape (picture 3) [20].

●Evaluation – Evaluation of infants with microtia and anotia includes audiologic testing and thorough examination to identify associated defects. In some cases, imaging may be warranted to detect associated abnormalities of the external auditory canal, middle, and inner ear [21].

Appropriate consultations in the neonatal period include a clinical geneticist, pediatric audiologist, pediatric otolaryngologist, or a pediatric plastic surgeon. Although surgical intervention for unilateral defects seldom is undertaken until the child reaches 6 to 10 years of age, these consultations are important to identify other abnormalities and to provide reassurance for the families. Long-term management may include audiology, particularly if there is associated external auditory canal atresia (aural atresia). Amplification devices are beneficial for children with bilateral hearing loss [22]. (See "Hearing loss in children: Treatment".)

●Surgical repair – A minor abnormality of the pinna may not require correction. When the pinna is grossly deformed or absent, reconstruction often is warranted for cosmetic reasons. Most repairs are undertaken when the child is 6 to 10 years of age, after the pinna has reached 80 percent of its adult size.

Microtia repair is complex and often requires several stages [23]. A cartilage framework, usually derived from costal cartilage, is created and anchored beneath the skin of the mastoid area. Once it is well attached to surface skin, a postauricular crease is created in a second operation. Additional procedures often are employed for "fine-tuning." Even in the best hands, the complex structure of the external ear is difficult to duplicate, and cosmetic results vary widely.

Some experts have advocated the use of synthetic auricular frameworks given the difficulty of creating an aesthetically pleasing auricle from autologous cartilage. Research into customized three-dimensional scaffolds is ongoing [24]. The placement of a prosthetic auricle anchored with an osteointegrated peg remains an option [25].

Lop or outstanding ear — Protruding ears usually result from incomplete formation of the antihelical fold (picture 4). This abnormality has no functional significance. However, children with protruding ears sometimes are teased or bullied by their peers. If the protrusion is not severe, non-surgical splinting in the neonatal period may correct the defect [26]. For more severe cases, otoplasty can be performed later in childhood, typically when the child is four to six years old. The most common procedures involve the excision of skin from the posterior surface of the auricle and the placement of permanent sutures to reposition the ear and create an antihelical fold [27]. These techniques provide high rates of patient and family satisfaction [28]. Complications occur in 0 to 8 percent of otoplasties and include hematomas, perichondritis, suture extrusion, and keloid formation. Residual deformity and asymmetry of the two ears may occur [29].

Preauricular pits — Preauricular pits are small indentations located anterior to the helix and superior to the tragus of the ear (bilateral in 25 to 50 percent of cases) (picture 5). They are quite common, noted in approximately 1 percent of white, 5 percent of black, and 10 percent of Asian children [30]. The prevalence is considerably higher in some family lineages [30]. Associated congenital anomalies occur in approximately one-third of the sporadic cases [31].

Infants with preauricular pits should have formal audiologic evaluation [32]. The risk of permanent hearing loss in children with preauricular pits or tags is five times that of the general population [32]. (See "Hearing impairment in children: Evaluation", section on 'Formal audiology'.)

The incidence of renal anomalies in patients with isolated preauricular pits does not differ substantially from that in the general population [33,34]. (See 'Association with renal anomalies' below.)

Preauricular pits may be the first indication of branchio-oto-renal (BOR) syndrome, one of the most common causes of hereditary hearing loss (picture 6). BOR is an autosomal dominant syndrome; most cases are due to mutations in the EYA1 and SIX1 genes [35]. It is characterized by sensorineural hearing loss (SNHL), preauricular pits, branchial cysts or tracts, malformed ears, and renal anomalies, including renal dysplasia and bifid renal pelvises [36]. (See "Renal hypodysplasia", section on 'Branchio-oto-renal syndrome'.)

Preauricular pits do not require surgery unless they become repeatedly infected or discharge squamous material (picture 5) [30]. If surgery is performed, it should include excision of the pit, the squamous-lined cyst usually present beneath the skin (picture 7), and the cartilage at the root of the helix en bloc to avoid recurrence (picture 8) [37].

Accessory auricular appendage/preauricular tag — Accessory appendages composed of skin, subcutaneous fat, and/or cartilage may occur near the auricle or anywhere along the anterior border of the sternocleidomastoid muscle (picture 9). When they occur in the preauricular area, they are called preauricular tags.

Accessory auricular appendages and preauricular tags usually are removed in childhood for cosmetic purposes. Excision with plastic surgical closure provides better results than neonatal ligation with suture or rubber bands because the lesions typically extend into subcutaneous tissue planes [38].

Children with accessory auricular appendages may have associated unilateral hearing loss and should undergo newborn hearing screening [32]. (See "Screening the newborn for hearing loss".)

Accessory auricular appendages, particularly at the tragus, with or without associated microtia may appear as part of the oculo-auriculo-vertebral spectrum (also called Goldenhar syndrome, hemifacial microsomia, facio-auriculo-vertebral spectrum, and first and second branchial arch syndrome). Preauricular tags may be seen in other genetic syndromes (eg, Townes-Brocks syndrome, branchio-oto-renal syndrome) [2,14]. (See "Syndromes with craniofacial abnormalities", section on 'Craniofacial microsomia'.)

Ear anomalies in CHARGE and DiGeorge syndromes — The external ear may have a characteristic appearance in these syndromes:

●CHARGE syndrome – In children with CHARGE syndrome (coloboma, heart defects, atresia choanae, retardation of growth and/or development, genital and/or urinary abnormalities, ear abnormalities and deafness), the ears are typically asymmetric between sides, have reduced vertical height, and have a cup-shaped, wide helix (picture 10) [39].

●DiGeorge syndrome – Children with DiGeorge syndrome (also called 22q11.2 deletion syndrome or velocardiofacial syndrome) typically have a thick/overfolded helix and slightly low set ears [40]. (See "DiGeorge (22q11.2 deletion) syndrome: Clinical features and diagnosis".)

Aural atresia (external auditory canal atresia) — Failure of complete invagination of the external auditory canal results in an absent or stenotic ear canal and improper formation of the eardrum. Failure of recanalization results in membranous stenosis or atresia (picture 11).

Formation of the malleus and incus occurs at the same time as invagination of the external auditory canal. Thus children with aural atresia commonly have associated abnormalities of these ossicles, particularly fusion of the incus and malleus to each other or to atretic bone in the auditory canal.

Children who have unilateral external auditory canal atresia may not require surgery if the pinna is well formed and the contralateral ear has normal hearing [41]. The use of a bone-conduction/bone-anchoredhearing aid in a child with unilateral aural atresia remains controversial. A bone-anchored aid may decrease head-shadow effect (ie, difficulty hearing sounds coming from the direction of the affected side), but does not improve localization of sound [42]. Amplification is important if the child has hearing loss in the contralateral ear.

Many children with external auditory canal atresia have normal middle ears and can develop acute otitis media (AOM). Children with aural atresia should be treated with antibiotics if AOM is suspected clinically, because the diagnosis cannot be confirmed through otoscopy [22,43]. (See "Acute otitis media in children: Treatment", section on 'Initial antimicrobial therapy'.)

Children with unrepaired aural atresia/stenosis also have a risk of developing middle ear cholesteatoma. Persistent ear pain or fever may be the only clues to this condition, which is diagnosed with computed tomography (CT) (image 1). (See "Cholesteatoma in children".)

Bilateral aural atresia is associated with a maximal conductive hearing loss (approximately 60 dB) and requires early intervention. Children with bilateral aural atresia should be fit with a bone-conduction hearing aid within weeks of birth to assist with early language acquisition [44]. (See "Hearing loss in children: Treatment", section on 'Bone conduction hearing devices'.)

Surgery to correct aural atresia usually is performed toward the end of the first decade of life, when substantial mastoid development has occurred. Surgery entails the creation of a new ear canal and ear drum, providing a skin lining for the canal and drum, and mobilizing or repositioning the ossicles to allow transmission of sound. Surgery is more likely to be successful if extensive pneumatization of the mastoid air cells is present and the stapes is mobile. One of the major risks of this surgery is injury to the facial nerve or the dura of the middle cranial fossa. Thus, the procedure should be performed by an experienced otologist and with electrophysiologic facial nerve monitoring. Other complications include chronic myringitis, prosthesis displacement, SNHL, soft-tissue stenosis and tympanic membrane lateralization [45]. Revision surgery is required in approximately 25 percent of cases [45]. The new ear canal often requires cleaning and care for life [46]. The bone-anchored hearing aid is an alternative for patients who are not candidates for aural atresiaplasty because of unfavorable anatomy or personal preference [47]. Devices that avoid a transcutaneous peg may improve patient acceptance of bone-anchored aids [48].

Population Reference No. 2 Policy Statement

For individuals with trauma to the ear structures causing significant incapacity. Interventions of interest are repair of injury sustained to areas of the ear. Comparators of interest are repair of injury or trauma. Relevant outcomes include overall quality of life, functional outcomes and symptoms.

Population Reference No. 3 Policy Statement

For individual with minor cosmetic complaints. Interventions of interest are repair of non functional anatomy or the external ear. Comparators of interest are cosmetic repair to restore anatomy. Relevant outcomes include overall quality of life, Functional, outcomes and Symptoms.

Supplemental Information

Not applicable

Practice Guidelines and Position Statements

Not applicable

Medicare National Coverage

Billing and Coding Guidelines for Cosmetic and Reconstructive Surgery LCD The following procedures may be considered reconstructive or cosmetic, 69300

Otoplasty. Cosmetic procedures and/or surgery are statutorily excluded by Medicare. These services will be denied as non-covered. Noncovered procedures

do not need to be billed to the Contractor. If the beneficiary requests a claim be submitted for a cosmetic procedure, then use the billing instructions below to

receive a non-covered cosmetic denial. See WPS LCD L34698 for coverage of the services that are reconstructive and therefore, medically necessary.

Cosmetic surgery can be defined as a procedure that is performed to reshape normal structures of the body in order to improve the patient's appearance and

self-esteem. These procedures can be performed for medically necessary or cosmetic reasons. See WPS LCD L34698 for the criteria for medically necessary

services. Information below is to assist with billing for these services when they are performed for cosmetic reasons.

References

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69.Lee D, Bluestone CD. The Becker technique for otoplasty: modified and revisited with long-term outcomes. Laryngoscope 2000; 110:949
 

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Policy History