CHAPTER I

INTRODUCTION

Microtia is an incomplete or abnormally formed ear. It literally comes from

the words “micro” (small) and “otia” (ear). Microtia may affect one side only
(unilateral) or affect both ears (bilateral).1 Microtia occurs in approximately 0.03% (1
in 10,000–20,000) of live births, and it is estimated that half of these patients have
associated congenital syndromes. Certain populations, such as the Navaho Indians
and the Japanese, may have higher incidences of microtia deformities appear to have
a right-ear preponderance and affect boys more often than girls at roughly a 2.5:1
ratio. Unilateral cases out number bilateral cases by a 4:1 ratio. Despite this relatively
low incidence, microtia is one of the more common congenital malformations
presented to otolaryngologists. Auricular reconstruction of the microtia deformity is a
complex and labor-intensive process that requires a great deal of preoperative
planning and preparation.2

The child born with a malformed ear faces a lifelong hearing and
communication impairment along with the social stigma of a facial deformity.
Frequently, there are additional anomalies, such as facial and skeletal deformities.
There may be dysfunction of associated neural pathways. Associated disturbances of
the vestibular system may cause developmental motor delay. Additionally, there are
psychological factors to be considered, including parental guilt, peer ridicule, and the
shame of “being different.” These hearing-impaired children face educational and
economic challenges that may limit their achievements and opportunities.3

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 CHAPTER II

OVERVIEW

A. Embriology
A review of embryology allows a better understanding of the pathophysiology
of microtia. The anatomy of the microtic ear is similar to the anlage seen in the 6-
week embryo. Microtia often is associated with atresia or absence of the external
auditory meatus, suggesting an arrest of development.2
During the sixth week of gestation, the external ear begins to develop around
the dorsal end of the first branchial cleft. On either side of the cleft lies the first
(mandibular) and second (hyoid) branchial arches. The auricle arises from these
arches as six small buds of mesenchyme, known traditionally as the six hillocks of
His. The mandibular arch gives rise to hillocks 1 through 3, and the hyoid arch gives
rise to hillocks 4 through 6. Traditionally, it is thought that the derivation of specific
auricle components comes from certain hillocks (ie, hillock 1 becomes the tragus,
hillocks 2 and 3 form the helix, hillocks 4 and 5 form the antihelix, and hillock 6
forms the lobule). Other theories suggest that the hyoid arch contributes
approximately 85% of the auricle, and that most of the central ear is formed from
hillocks 4 and 5, whereas the tragus is formed from hillocks 1 through 3. The lobule
is the last component of the external ear to develop. The concha is derived from the
ectoderm forming the EAC. The auricle begins in the anterior neck region, then
migrates dorsally and cephalad as the mandible develops during gestational weeks 8
through 12, and lies in its relative adult location by gestational week 20.2,4
During the first and second month gestation, the external auditory meatus
develops from the first branchial groove between the mandibular and hyoid arches.
At 4 to 5 weeks gestation, a solid epithelial nest forms at the meatus, and contacts the

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endoderm of the first pharyngeal pouch. Mesoderm interrupts the contact between the
endoderm and ectoderm. At 8 weeks, the cavum conchae invaginates, forming the
primary meatus, which becomes surrounded by cartilage and eventually becomes the
fibrocartilaginous portion of the EAC. This groove deepens and grows toward the
tympanic cavity and comes into contact with the epithelium of the first pharyngeal
pouch. A solid epidermal plug extends from the primary meatus to the primitive
tympanic cavity to form the meatal plate. This plug of tissue recanalizes during the
twenty-first week, forming the primitive EAC, and by the twenty-eighth week, only
the most medial ectoderm remains, forming the superficial layer of the tympanic
membrane. The ectoderm lines the bony portion of the EAC.2,4

B. Microtia
Definition

Microtia is an incomplete or abnormally formed ear, it comes from the words

“micro” (small) and “otia” (ear).1 Microtia is a congenital anomaly, characterized by
a small, abnormally shaped auricle (pinna). It is usually accompanied by a narrow,
blocked or absent ear canal. Microtia can occur as the only clinical abnormality or as
part of a syndrome.5

Etiology
Although the etiology of microtia is poorly understood, both environmental
and genetic factors have been implicated. Microtia can coexist with several known
syndromes associated with inherited defects or acquired embryopathies owing to
intrauterine infection (rubella, syphilis), ischemic injury (hemifacial microsomia), or
toxin exposure (thalidomide, isotretinoin). The genetic basis of external and middle
ear anomalies generally remains poorly characterized.3
There are many risk factors for microtia, such as gestosis, anemia, race, high
maternal or paternal age, and multiple births. In a clinical analysis of 592 patients
with microtia in Japan, 28% of the patients' mothers had a cold, imminent abortion,

3
gestosis or anemia during their pregnancy. Maternal acute illness, such as influenza,
during first trimester of pregnancy was the major risk factor for microtia in Latin
America. Mothers with chronic type I diabetes are at significantly higher risk for
having a child with microtia. Microtia children usually have normal birth weight, but
low weight birth is more common than in healthy children. Certain medications such
as isotretinoin, a widely used dermatologic drug, or mycophenolate mofetil (MMF)
taken by the mother during pregnancy have also been implicated as microtia-
predisposing factors.5
Diagnosis
Usually, the diagnosis is evident on inspection of the external ear. Depending
on the degree of the abnormality, the microtic ear may be classified in three grades. 3
Grade I microtia represents a minor malformation, with the auricle being smaller than
normal but with all parts discernible. In grade II microtia, the auricle is represented
by a curving or vertical ridge of tissue. In grade III microtia, any resemblance to an
auricle is lost, and only a small rudimentary soft tissue structure is present. Grade II
or III microtia is common, and inner ear function is usually normal. 6 The caliber of
the external auditory canal should be graded as normal, stenotic (i.e., mild or severe),
blindly ending, or completely atretic. If possible, the tympanic membrane is
examined otoscopically. Displacement of the malleus handle (usually anteriorly) or a
bony shelf extending from the posterior canal wall may exist. The otologist also
should consider evaluation of the function of other organ systems to detect any
coexisting developmental defects.3,6
Brainstem response audiometry should be performed neonatally in all children
born with either microtia or atresia. In cases of microtia with fairly normal canals, an
ossicular malformation producing a conductive hearing loss may be present.
Temporal bone CT is performed near the time of operation. Radiographic studies on
infants are usually not recommended because the information is rarely applicable to
immediate rehabilitative plans.6
Treatment

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 Microtia repair, especially for unilateral cases, is typically delayed until
children reach six-seven years of age.6 The surgeon must balance concerns regarding
the psychological impact of the deformity with having sufficient cartilage to carry out
the optimal reconstruction in the fewest number of surgeries. In microtia patients
requiring major external ear reconstruction, it is reasonable for the reconstructive
surgeon to operate first. This ensures a virgin field without scars or compromised
blood supply, optimizing survival of the implanted auricular framework. The overall
cosmetic result should also be better without the restriction of having to reconstruct
the auricle around a bony canal drilled in the temporal bone. Typically, the otologic
surgery is performed midway through the multistage microtia repair, after the
auricular framework has been implanted and the lobule transposed but before the
tragus is reconstructed and the auricle is elevated from the side of the head. Although
the reconstructed auricle may not be centered exactly over the created bony canal, it
can be repositioned with appropriate undermining so that the meatus and external
canal are aligned. Optimal reconstruction requires costal cartilage of sufficient size
and shape to carve the key framework details and maintain the strength to display
these details through the overlying skin envelope. While some surgeons begin the
reconstruction when patients are aged as young as 5-6 years, clearly better results can
be obtained if the reconstruction is performed when patients are aged 8-10 years or
older.1,6
If congenital aural atresia is also present, drill-out procedures are usually
postponed until age seven at the earliest although some surgeons prefer not to
consider such surgeries until after reconstruction is completed so as to minimize any
potential disruption to the vascular supply to the repaired auricle. Reconstruction of
the external ear can be performed in 1 of 3 fashions: prosthetic replacement,
reconstruction with a prosthetic framework, or local tissue and/or flap coverage or
reconstruction with an autologous framework.3,7

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 CHAPTER III
CASE REPORT

A. Identity
Name : Baby SF
Sex : Female
Age : 6 Months
Address : Jeruklegi, Cilacap
MR : 01.52.54.82
B. Alloanamnesis
Main Complaint : Right ear shape is not normal
Present history illness :
The patient came to the ENT clinic of RSUP Dr. Sardjito and complained
that right ear shape of her baby was not normal. The right ear didn’t look like
as normally ear, it looked smaller than the left one. She also complained that
there was no ear canal of the right ear. There was no complaint of nose and
throat.
Past history illness :
History of pregnancy : During the pregnancy the mother often drank some
herbal baverage (jamu gendong). She also took vitamins given by midwife.
History of hypertension, bleeding, seizures, trauma, and severe illness during
pregnancy were denied.
History of birth: baby was born with sufficient gestational age, normal
delivery handled by midwives, birth weight: 3000gr, the baby cried directly.
Family history illness:
There was no family who suffered from that kind of disease in the family.
There was no family member who had congenital anomalies.

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C. Physical examination
General status :
Compos mentis, good nutritional status
Vital signs:
Heart rate : 100x/menit
RR : 30 times/minute
Temperature : 37,3 ºC
ENT Examination
Right Ear : Auriculae is almost absent, vertical sausage-shaped skin
remnant and there is no external auditory canal of auris dextra
Left Ear : Auriculae and external auditory canal are within normal limit,
tympanic membrane is within normal limit
Nose : Rhinoscopy anterior is within normal limit, rhinoscopy
posterior is difficult to assess
Oropharynx : within normal limit
Indirect laryngoscope: difficult to assess
Additional examination:
BERA (Brainstem Evoked Response Audiometry) test :
AD : severe neural hearing loss
AS : moderate neural hearing loss
D. Diagnose
AD Microtia grade III
E. Therapy
Education
F. Problem
Prognosis
G. Plan
BERA test 6 months later
Consul to Growth & Developmental Pediatric Departement

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 CHAPTER IV
DISCUSSION

Microtia is a congenital anomaly, characterized by a small, abnormally shaped

auricle (pinna). It is usually accompanied by a narrow, blocked or absent ear canal.
Microtia can occur as the only clinical abnormality or as part of a syndrome.5 In grade
III microtia, any resemblance to an auricle is lost and only a small rudimentary soft
tissue structure is present.6 According to physical examination of the right ear
patient’s auricle which is almost absent and there is no external auditory canal, this
condition is diagnosed as microtia grade III. Congenital microtia, a small and
misshape auricle and aural atresia, an absent external auditory canal (EAC) are two
developmental malformations that present a unique challenge when coexistent
because the EAC and auricle are closely related in embryonic development, nearly all
children with grade III microtia have canal atresia.3,7
Patients with unilateral microtia typically have normal hearing in the
contralateral ear, although this should be confirmed with an audiogram. If the child is
younger than 7 months old, we obtain an auditory brain response or an evoked
otoacoustic emission examination. In children 7 months to 2 years old, we first obtain
behavioral audiograms using visual-reinforced audiometry; if this test is abnormal,
we proceed with the auditory brain response or evoked otoacoustic emission. 2 The
BERA result of patient are moderate neural hearing loss for the left ear and severe
neural hearing loss for the right ear. There is no specific medication for the patient,
the treatment’s giving education to the parents about their child’s condition and we
plan to do BERA test 6 month later to evaluate hearing status.
Congenital ear malformations are not detected readily before birth, and may
cause psychosocial trauma to the parents and family. Counseling the family members
is an important part of the management of these conditions. The expectations of the

8
parents and the reality of the problem may not coincide. It is important to
communicate to the family that although microtia reconstruction will improve the
appearance of the auricle, the ear may never look normal.

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 CHAPTER IV
CONCLUSION

Have been reported a female baby, 6 months aged, came to ENT Clinic
Sardjito General Hospital and was diagnosed as microtia auricle dextra grade III. We
plan to do evaluation and auditory brainstem response test 6 month later and give her
parents education about the deformities.

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 REFERENCES

1. Lotz et al. Microtia and Ear Anomalies in Children. Department of Plastic and
Maxillofacial Surgery, The Royal Children’s Hospital, Melbourne, Australia.
2. Murakami CS, Quatela VC. Reconstruction Surgery of The Ear: Microtia
Reconstruction. In Cummings: Otolaryngology, Head & Neck Surgery. 4th
ed. 2005.
3. Parisier SC, Fayad JN, Kimmelman CP. Microtia, Canal Atresia, and Middle
Ear Anomalies. Dalam Ballenger’s Manual of Otorhinolaryngology Head and
Neck Surgery. London: BC Decker. 2002. P: 353-362.
4. Kelley PE, Scholes MA. Microtia and Congenital Aural Atresia. Otolaryngol
Clin N Am. 2007;40: 61–80.
5. Alasti F, Camp GV. Genetics of Microtia and Associated Syndromes. Journal
of Medical Genetics. 2009;46: 1-23.
6. Lambert PR. Congenital Aural Atresia. Dalam Bailey BJ, Johnson JT, Head &
Neck Surgery-Otorhinolaryngology. 4th ed. Philadelphia: Williams &
Wilkins. 2006.
7. Pham V. Microtia: With An Emphasis On Reconstructive Options. Grand
Rounds Presentation, UTMB, Dept. of Otolaryngology. 2010.

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 MICROTIA

1. ANAMNESIS
- What’s the chief complaint?
- How is the shape of the pinna?
- Is there any external auditory canal?
- Is there any severe illness during the pregnancy?
- Did the mother take herbal baverage/jamu?
- Is there any trauma during the pregnancy?
- Is it enough gestational age?
- How much is baby birth weight?
- How was the delivery?
- Did the baby cry directly after delivery?
2. Physical Examination
- Inspection
- Palpation
3. Differential Diagnosis
- Microtia
- Anotia
4. Additional Examination
- Audimetric evaluation
- CT-Scan Temporal bone
5. Treatment
- Education
- Reconstruction surgery

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